Hidradenitis suppurativa: Treatment

Author:
John R Ingram, MD, PhD
Section Editors:
Robert P Dellavalle, MD, PhD, MSPH
Mark V Dahl, MD
Deputy Editor:
Abena O Ofori, MD

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is
complete.
Literature review current through: Oct 2018. | This topic last updated: Jul 02, 2018.

INTRODUCTION — Hidradenitis suppurativa (HS) is a chronic, painful, follicular

occlusive disease that affects the folliculopilosebaceous unit, mainly but not exclusively in
intertriginous axillary, groin, perianal, perineal, genital, and inframammary skin. The
clinical course is highly variable, ranging from relatively mild cases characterized by the
recurrent appearance of papules, pustules, and a few inflammatory nodules to severe cases
demonstrating deep fluctuant abscesses, draining sinuses, and severe rope-like scars
(picture 1A).

The management and prognosis of HS will be reviewed here. Information on the

pathogenesis, clinical features, and diagnosis of HS and a detailed discussion of the surgical
techniques used in the treatment of HS are provided separately. (See "Hidradenitis
suppurativa: Pathogenesis, clinical features, and diagnosis" and "Surgical management of
hidradenitis suppurativa".)

PRINCIPLES — The pain, odor, drainage, and disfigurement caused by HS profoundly

affects quality of life [1]. Patients often experience feelings of sadness or depression related
to the disease, and feelings of shame may contribute to self-imposed social isolation [2-4].
Diagnostic delay, which averages seven years, combined with the significant physical and
emotional toll of HS, may lead to patient frustration with medical care [5].

Treatment goals — Untreated, HS can remain active for many years after onset, which is
typically in early adulthood. Interventions for HS target one or more of three major goals:

●To reduce the frequency of new lesions, minimizing pain and suppuration

●To prevent disease progression by limiting the formation of scarring

●To treat existing lesions and scarring, a goal which may require a combination of medical
and surgical intervention
More than 50 interventions exist for treatment of HS, including patient self-management
strategies, topical therapy, oral systemic agents, biologic therapies, surgery, and laser and
light interventions. Disease severity, patient tolerance of specific agents, comorbidities, and
treatment cost and availability guide treatment choices. (See 'Treatment' below.)

Staging systems — The Hurley clinical staging system is frequently utilized to describe the
severity of HS [6]. In accordance with this system, HS is divided into three stages:

●Stage I – Abscess formation (single or multiple) without sinus tracts and

cicatrization/scarring (picture 2)

●Stage II – Recurrent abscesses with sinus tracts and scarring, single or multiple widely
separated lesions (picture 3)

●Stage III – Diffuse or almost diffuse involvement, or multiple interconnected tracts and
abscesses across the entire area (picture 1A-B)

Outcome measure instruments — The Hurley staging system has insufficient

responsiveness to change to be used as a clinical study outcome measure. The Sartorius
scale [7] and modified Sartorius scale [8,9] have been used in clinical trials, and newer
assessment tools, such as the Hidradenitis Suppurativa Clinical Response (HiSCR) and
Severity Assessment of Hidradenitis Suppurativa Score, have been developed and validated
[10,11]. The Hidradenitis Suppurativa Core outcomes set International Collaboration
(HISTORIC) is developing a core domain set for HS trial outcomes [12].

In a busy clinic setting, other instruments are useful for quickly assessing disease severity.
Patients can complete a quality-of-life instrument, such as the Dermatology Life Quality
Index or Skindex, and a pain visual analogue scale (form 1) [13,14]. They can also be asked
to record the number of new or recurrent lesions occurring in the preceding month.

TREATMENT — The multifaceted clinical features of HS and the unpredictable course of

the disease make a uniform approach to treatment challenging. (See 'General approaches'
below and 'Hurley stage I (mild disease)' below and 'Hurley stage II' below and 'Hurley
stage III (severe and refractory disease)' below.)

General approaches — Regardless of disease severity, attention to patient education and

support, patient self-management, management of comorbidities, and pain management
may be beneficial.

Education and support — Because HS is a psychologically distressing disorder that can

have a major impact on patient quality of life, patient education and support are essential
components of management. The clinician should inquire about the impact of the disease
on the patient's quality of life and assess for symptoms of depression [15]. (See "Screening
for depression in adults".)
Patients should be informed that the disease is neither contagious nor due to poor hygiene.
Patients should also be offered resources for psychologic support [3], such as contact
information for local HS patient societies.

Self-management — Patient self-management is an important part of the approach to HS.

Patients should be offered individualized self-management strategies.

●Avoidance of skin trauma – Factors that promote skin maceration and follicular trauma
may contribute to worsening of HS due to the promotion of inflammation, follicular
occlusion, and follicular rupture. We advise patients to wear loose, light clothing and to
actively avoid excessive heat, friction, and shearing trauma. Washing with loofahs or
brushes should be avoided to prevent unnecessary skin trauma and irritation.

●Dressings – Whether dressing untreated but actively draining lesions or the postoperative
wound, dressings should be selected that minimize skin trauma. To prevent absorbent
dressings from sticking to the wound, simple white petrolatum can be applied. Adhesive
tape should be avoided, and instead, elastic fishnet dressings can be customized to hold
absorbent material in place.

●Smoking cessation – Smoking is commonly associated with HS. The case for smoking as
a causative factor is strengthening [8,16-19], and documentation of improvement in HS
after smoking cessation has appeared in a few case reports [20]. Despite the fact that the
impact of smoking cessation on HS is untested by clinical trials, this observed association,
the observed increase in risk for cardiovascular-associated death among patients with HS
compared with patients without the disease [21], and the overall negative impact of
smoking on health lead us to routinely discourage smoking and other nicotine use. Ideally,
smoking cessation support should be offered as part of the multidisciplinary approach to
HS management. (See "Hidradenitis suppurativa: Pathogenesis, clinical features, and
diagnosis", section on 'Associated factors' and "Overview of smoking cessation
management in adults" and "Behavioral approaches to smoking cessation".)

●Weight management – As with smoking, excess weight has been linked to HS, but a
causative association between weight and HS has not been definitively established
[8,16,17]. Factors related to excess weight, such as skin occlusion, skin friction, shearing
forces on skin, hyperinsulinemia and other hormonal changes that can occur in association
with excess weight, and dietary factors appear to be contributing factors in HS. Thus, we
encourage and support weight management in our HS patients. In addition, we sometimes
prescribe metformin, an antidiabetes drug that may be beneficial for HS and may have a
modest effect on weight loss [22,23]. (See "Hidradenitis suppurativa: Pathogenesis, clinical
features, and diagnosis", section on 'Associated factors' and "Obesity in adults: Overview of
management" and 'Metformin' below.)

Data are emerging regarding the effect of substantial weight loss in obese HS patients. In a
retrospective survey of 45 Danish patients who had undergone bariatric surgery, 35
achieved substantial weight loss, defined as at least a 15 percent reduction in baseline body
mass index, and, of these, 17 experienced resolution of their HS, 7 were improved, and 11
had unchanged or worsened disease [24]. Removal of excess skin following substantial
weight loss may be required to prevent disease exacerbations related to increased frictional
effects.

●Antiseptics – Topical antiseptic washes such as chlorhexidine 4% may be helpful for HS.
Chlorhexidine can be used for showering, beginning with once per week use and increasing
up to once daily as tolerated. In addition, some patients find emollients containing
benzalkonium chloride, an antimicrobial agent, to be helpful.

Management of comorbidities — Patients with HS may be at increased risk for alcohol

dependence, arthropathies, autoimmune conditions, cardiovascular risk, diabetes, drug
dependence, dyslipidemia, follicular syndromes, hypertension, lymphoma, malignancies,
metabolic syndrome, nicotine addiction, obesity, polycystic ovarian syndrome, psychiatric
and psychologic disorders, rheumatologic conditions, and thyroid disease [25,26]. (See
"Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis", section on
'Associated disorders and syndromes'.)

Multidisciplinary management of comorbidities can improve patient outcomes.

Recommendations from a multispecialty working group for the management of
comorbidities, including cardiovascular risk factors, excess weight, inflammatory bowel
disease, inflammatory joint disorders, psychologic disorders, alcohol use, and tobacco use,
in patients with HS have been published [27]. The document provides guidance for
dermatologists for patient assessment and referral and reviews the implications of
treatments for HS on comorbidities. We agree with the importance of assessing for
comorbidities on a yearly basis and support multidisciplinary involvement in the
management of comorbidities.

Pain management — Pain from HS nodules and abscesses may cause sleep disturbance,
limit function, and induce psychologic distress. Nonsteroidal anti-inflammatories can be
used to treat both pain and inflammation. Additional analgesia, including opioid analgesia,
may be needed. (See "Overview of the treatment of chronic non-cancer pain".)

Hurley stage I (mild disease) — Local therapy combined with the general approaches
described above is the preferred treatment of mild HS (picture 2). (See 'General approaches'
above.)

Useful local measures include topical clindamycin, intralesional corticosteroid injections,

punch debridement, and topical resorcinol. When these measures are insufficient, oral
tetracyclines may be beneficial.

Topical clindamycin — Topical clindamycin is often utilized as a first-line therapy for mild
HS. A randomized three-month trial supported its efficacy for mild inflammatory lesions
and demonstrated the relative safety and tolerability of this regimen [28]. Patients with mild
HS were treated with twice-daily applications of clindamycin 1% solution (13 patients) or
vehicle (14 patients). Treatment efficacy was assessed with an unvalidated scoring system
that was designed to assess the amount of disease. The trial found that the cumulative
disease burden score improved to a significantly greater degree in patients treated with
topical clindamycin than in patients who were given placebo at all study time points (one,
two, and three months after the start of treatment). Treatment with clindamycin was well
tolerated; two patients treated with clindamycin 1% solution and three patients treated with
vehicle solution reported slight burning sensations at the sites of treatment.

Intralesional corticosteroids — Intralesional corticosteroid therapy is often useful as an

adjunctive therapy for reducing symptoms of HS. The intent of treatment is to accelerate
the resolution of early, painful inflammatory lesions. A prospective series of 36 HS patients
designed to assess the effect of triamcinolone 10 mg/mL (volume range 0.2 to 2 mL)
injected into an acute nodule or abscess supports this approach [29]. Mean pain, measured
on a visual analogue scale (VAS) from 0 to 10, where 10 represents greatest pain,
decreased from 5.5 pretreatment to 1.1 at follow-up after a mean of seven days. Significant
differences in pain VAS were found between days 0 and 1 and also from day 1 to day 2
after the injection. (See "Intralesional injection".)

Punch debridement — Performance of punch debridement (partial deroofing) to evacuate a

new inflamed nodule that is centered around a single folliculopilosebaceous unit (FPSU)
can be effective for eliminating new lesions. Simple incision and drainage are usually not
performed because incised nodules usually recur. (See "Surgical management of
hidradenitis suppurativa", section on 'Punch debridement' and "Surgical management of
hidradenitis suppurativa", section on 'Role for I&D'.)

Topical resorcinol — Resorcinol is a topical chemical peeling agent with keratolytic and
anti-inflammatory properties that is used by some clinicians for HS. Topical 15% resorcinol
in a proprietary cream base is applied directly to inflammatory nodules.

In an open study of 12 patients with Hurley stage I or stage II HS who applied topical 15%
resorcinol once to twice daily primarily during disease flares, all patients experienced a
reduction in pain and a reduction in duration of painful abscesses [30]. The expected
adverse effect of local desquamation occurred in all patients. Recurrences may follow
discontinuation of the medication.

Patients can be instructed to apply a thin film of resorcinol 15% cream directly to a new
inflamed nodule twice daily. Resorcinol is not applied to the entire region. As improvement
occurs, the frequency of application may be tapered to once daily and application to the site
of the inflamed nodule can be discontinued upon resolution.

Systemic antibiotics — Short courses (eg, seven days) of penicillin-type antibiotics do not
appear to alter the natural history of an acute HS lesion [31]. In addition, multiple short
antibiotic courses may promote bacterial resistance. A strategy endorsed by the European
HS guidelines is to give a more prolonged course of an oral tetracycline such as
lymecycline or doxycycline, with the treatment goal to prevent or reduce the frequency of
new lesions [32]. (See 'Oral tetracyclines' below.)

Hurley stage II — Hurley stage II HS is characterized by inflammatory nodules, sinus

tracts, and scarring (picture 3).
Antibiotic therapy — The mechanism by which antibiotic therapy improves HS has not
been definitively determined. Antibiotics may help to control skin bacterial load, however;
the anti-inflammatory effects associated with some antibiotics may also play a role [33].

First-line antibiotic therapy

Oral tetracyclines — Tetracyclines are a key treatment for mild to moderate HS, in part
because of their favorable adverse effect profile. A common regimen for adults is 100 mg
of doxycycline given once to twice daily. Alternative tetracycline regimens include
lymecycline (408 mg once or twice daily), tetracycline (500 mg twice daily), and
minocycline (100 mg once or twice daily). Treatment is generally continued for several
months.

In a trial, 46 patients with stage I or II HS were randomly assigned to treatment with oral
tetracycline (500 mg twice daily) or topical 1% clindamycin solution (applied twice daily)
[34]. Patients were treated for 16 weeks, and, while there was no difference between the
groups in terms of pain, number of lesions, or physician global assessment, there was a
greater improvement in patient global assessment in the oral tetracycline group.

Clindamycin and rifampin — Combination therapy with clindamycin and rifampin is an

option for patients who fail to respond to oral tetracyclines. Use of this regimen is based
upon uncontrolled studies [35-38]. In the largest study that evaluated this regimen, 116
patients with primarily Hurley stage I and II HS were treated with clindamycin (300 mg
twice daily) and rifampin (600 mg once daily) for 10 weeks [35]. Significant decreases in
the Sartorius score were observed, from a median of 28 points at baseline to 19 points at the
end of treatment, and 8 of the 70 patients (11 percent) who were available for the week 10
assessments achieved complete remissions (Sartorius score of 0).

In a retrospective study of 34 patients with stage I, II, or III HS who had failed various
other treatments, 16 (47 percent) achieved a complete response (defined as >75 percent
improvement) to clindamycin and rifampin administered via various treatment regimens
[36]. However, the long-term benefit of treatment after treatment cessation was variable. In
the study, 8 of 13 patients (62 percent) who responded completely to a regimen of
clindamycin 300 mg twice daily and rifampin 300 mg twice daily (including nine patients
treated for ≥10 weeks) relapsed after an average of five months. In a separate prospective
case series of 26 patients with HS, treatment with clindamycin (300 mg twice daily) and
rifampin (600 mg per day) for 12 weeks was associated with a clinical response (defined as
at least 50 percent clinical improvement) in 19 patients (73 percent) [38]. Relapse did not
occur in 7 of the 17 responders (41 percent) available for follow-up one year after
treatment.

Combination therapy with clindamycin and rifampin is usually reserved for patients who
are unresponsive to oral tetracyclines due to concern for drug-related adverse effects.
Gastrointestinal adverse effects of this treatment regimen are common [35,36]. While the
potential for Clostridioides (formerly Clostridium) difficile infection exists, this not been
highlighted as a major issue in the HS literature. Patients should also be advised that
rifampin causes orange discoloration of bodily secretions, and the effectiveness of
hormonal contraception may be reduced. Drug interactions due to rifampin should also be
reviewed. (See "Clostridioides (formerly Clostridium) difficile infection in adults: Clinical
manifestations and diagnosis" and "Clostridioides (formerly Clostridium) difficile infection
in adults: Epidemiology, microbiology, and pathophysiology", section on 'Antibiotic use'
and "Rifamycins (rifampin, rifabutin, rifapentine)", section on 'Adverse effects'.)

Other antibiotics — Dapsone, a sulfone drug with immunomodulatory and antibacterial

properties that is utilized for the treatment of multiple neutrophil-predominant skin
diseases, may be effective in mild to moderate HS, particularly in the early neutrophil-
mediated phase of new lesions [39,40]. In a retrospective study of 24 patients with Hurley
stage I to III HS who were treated with 50 to 200 mg daily of dapsone, six patients (25
percent) achieved clinically significant improvement and three patients improved slightly
(13 percent) [39]. None of the four patients with stage III disease improved with therapy. In
addition, dapsone (25 to 150 mg per day) was associated with reductions in disease severity
in a case series of five patients [40]. As with other medical agents, the disease often recurs
after treatment cessation [39,40].

Hemolysis is a common and expected adverse effect of dapsone therapy, and careful
laboratory monitoring for hematologic toxicity is necessary during treatment. It is prudent
to test for glucose-6-phosphate dehydrogenase (G6PD) deficiency prior to treatment to
avoid severe hemolysis in the G6PD-deficient population. Examples of additional adverse
effects of dapsone include methemoglobinemia (which manifests with headaches),
agranulocytosis, and a hypersensitivity reaction [41]. (See "Diagnosis and management of
glucose-6-phosphate dehydrogenase (G6PD) deficiency".)

Combination therapy with rifampin, moxifloxacin, and metronidazole appeared to be

beneficial for reducing disease activity in a retrospective study of 28 patients with
longstanding HS that was refractory to other treatments (short-course antimicrobials,
surgical drainage, and/or surgical excision) [42]. Patients with stage I or II HS appeared to
benefit most from this intervention.

Erythromycin and cephalosporins have also been used for long-term antibiotic therapy [43].

Oral retinoids — Acitretin, isotretinoin, and a newer oral retinoid, alitretinoin, have been
used to treat HS. Acitretin is the primary agent utilized [32]. Isotretinoin remains the gold
standard treatment for severe acne vulgaris and is often used to treat concomitant acne in
HS patients; however, it appears to provide only limited benefit for HS.

●Acitretin – A prospective uncontrolled study of 17 patients with HS treated with acitretin

(mean dose of 0.56 mg/kg/day) found a clinical response (at least 50 percent decrease in the
HS Severity Index score after six months of treatment) in 8 of the 14 patients who attended
for follow-up [44]. Only nine patients completed the planned nine months of therapy. The
primary reasons for withdrawal from the study were treatment inefficacy and side effects.

A retrospective study of 12 patients with recalcitrant Hurley stage II or III HS treated with
acitretin (mean dose 0.6 mg/kg daily) for 9 to 12 months with or without topical therapy
found that all patients improved and that nine patients achieved marked or complete
remissions [45]. The first initial signs of improvement were noted within approximately
two months. Improvement often persisted after treatment, with nine patients maintaining
responses for 6 to 45 months after the cessation of therapy.

●Isotretinoin – Studies of isotretinoin therapy have demonstrated improvement in relatively

low proportions of patients with HS. In a series of 88 patients treated with isotretinoin for
an average of eight months (mean daily dose 44 mg/day, range = 20 to 140 mg per day),
improvement was reported in only 14 patients (16 percent) [46]. In a retrospective study of
68 patients with various stages of HS who were treated for four to six months with
isotretinoin (mean daily doses of 0.5 to 0.8 mg/kg), 16 (24 percent) achieved clearing of
disease, and 25 showed lesser improvement [47]. All patients who cleared had disease that
was mild or moderate in severity.

●Alitretinoin – In a series of 14 patients with HS, treatment with alitretinoin (10 mg per
day) for 24 weeks produced improvement in 11 patients (79 percent), including 6 who
achieved significant improvement (at least 50 percent reduction in Sartorius score) [48].
Alitretinoin is not available in the United States.

Potential adverse effects of systemic retinoids include cheilitis, xerosis, hyperlipidemia, and
depression [49].

Retinoids are teratogenic, so use must be avoided in women who are at risk for pregnancy
and during pregnancy. Pregnancy should also be avoided for five weeks following
isotretinoin and alitretinoin treatment and for three years after acitretin. Thus, the use of
acitretin is typically avoided in women of childbearing-potential. In the United States,
isotretinoin can only be prescribed through the iPLEDGE program, an internet-based risk
management program. (See "Oral isotretinoin therapy for acne vulgaris", section on
'Isotretinoin safety'.)

Hormonal therapy — Androgens may contribute to the development of HS [33]. Examples

of antiandrogenic therapies that may improve the disease include cyproterone acetate, oral
contraceptive pills, spironolactone, and finasteride.

Cyproterone acetate was compared with norgestrel-containing oral contraceptive pills in a

randomized, double-blind crossover trial of 24 women with moderate to severe HS [50].
Patients were treated with 50 mcg of ethinyl estradiol (cycle days 5 to 25) and 50 mg of
cyproterone acetate (cycle days 5 to 14) for six months and ethinyl estradiol 50
mcg/norgestrel 500 mcg (cycle days 5 to 25) for six months. Although six patients dropped
out of the trial prior to completion due to drug intolerance or worsening of disease, both
treatment regimens were associated with clinical improvement, and no significant
difference in efficacy was detected. Overall, improvement occurred in 12 patients,
including seven patients who achieved complete remissions.

In addition to cyproterone acetate and oral contraceptive agents, spironolactone and

finasteride have been used for HS [51-55]. A retrospective chart review found that 16 of 29
women (55 percent) treated with various antiandrogenic drugs (cyproterone acetate
containing oral contraceptive, cyproterone acetate, and/or spironolactone) versus 6 of 23
women (26 percent) treated with oral antibiotics responded to treatment [51].

One retrospective case series of 20 women given spironolactone, most at a dose of 100 mg
daily, reported remission in 11 patients (55 percent) and clinical response in 17 patients (85
percent) after three months [52]. However, interpretation of these results is difficult because
five patients were started on concomitant minocycline 100 mg daily, and seven patients
received an oral contraceptive pill. Small case series have suggested benefit from
finasteride [53,54].

Hormonal therapy should not be given to pregnant women because of the risk for adverse
effects on the fetus. Finasteride is also contraindicated in women of childbearing potential.

Surgery — Surgery can be used for the treatment of individual nodules and sinus tracts that
occur in any Hurley stage of disease. Punch debridement (limited deroofing [unroofing]) of
fresh lesions or deroofing of single nodules and extensive sinuses is usually sufficient; wide
excision is typically reserved for Hurley stage III disease. (See "Surgical management of
hidradenitis suppurativa", section on 'Punch debridement' and "Surgical management of
hidradenitis suppurativa", section on 'Wide excision and reconstruction'.)

Hurley stage III (severe and refractory disease) — Both medical and surgical therapies are
utilized to prevent the development of new lesions and to treat existing lesions in patients
with refractory Hurley stage II HS and Hurley stage III HS (picture 1A-B). Although
extensive surgical intervention generally offers the greatest likelihood for the resolution of
active inflammation in the treated area [56], the procedure can be disfiguring and involve a
prolonged recovery time. In addition, extensive excision is not usually feasible in patients
with multiple affected skin regions. (See 'General approaches' above and 'Medical therapy'
below and 'Surgery' below and "Surgical management of hidradenitis suppurativa".)

Medical therapy — Patients who do not respond sufficiently to oral antibiotics, oral
retinoids, or hormonal therapies may benefit from biologic treatments, in particular,
adalimumab or infliximab. Ustekinumab has demonstrated benefit in small numbers of
patients.

The pharmacologic therapies utilized for severe and refractory disease are reviewed below.

TNF-alpha inhibitors — The tumor necrosis factor (TNF)-alpha inhibitors adalimumab and
infliximab have been utilized for the treatment of HS [57-64]. Additional studies are
necessary to compare the efficacy of these biologic therapies with each other and with other
therapies used for HS. Adalimumab is the only agent approved by the US Food and Drug
Administration (FDA) for the treatment of HS. Etanercept, another TNF-alpha inhibitor,
does not appear effective for HS [60].

●Adalimumab – Improvement in HS during weekly treatment with adalimumab has been

reported in randomized trials. The recommended dosing schedule for adults with HS is an
initial 160 mg subcutaneous dose (given at one time or split into two 80 mg doses given
over two consecutive days), then 80 mg on day 15, then 40 mg once weekly starting on day
29. Every-other-week dosing of adalimumab does not appear to be effective [60].

The FDA approval of adalimumab for moderate to severe HS was based upon the results of
two similar phase III randomized trials (PIONEER I [n = 307] and PIONEER II [n = 326]).
Patients with moderate to severe HS were randomly assigned either to adalimumab (40 mg
once weekly) or placebo for the initial 12 weeks (period 1) [61]. This was followed by a 24-
week phase (period 2) in which patients who received adalimumab in period 1 were
randomly assigned to adalimumab weekly, adalimumab every other week, or placebo.
Patients who received placebo in period 1 were reassigned to adalimumab weekly in
PIONEER I and to placebo in PIONEER II. Patients in PIONEER II were allowed to
continue oral antibiotic therapy at stable doses.

At week 12, more patients in the adalimumab groups achieved the Hidradenitis Suppurativa
Clinical Response (HiSCR) primary efficacy endpoint (≥50 percent reduction in the total
abscess and inflammatory nodule count with no increase in the abscess or draining sinus
count) than in the placebo groups (42 versus 26 percent in PIONEER I and 59 versus 28
percent in PIONEER II). In addition, in PIONEER II but not PIONEER I, adalimumab
treatment was associated with improvement in the secondary outcomes of lesion count,
pain score, and the modified Sartorius score for disease severity at week 12.

The response to adalimumab declined in period 2; among patients who responded to

adalimumab in period 1, no significant difference in clinical response rates was detected
between patients who received adalimumab versus placebo in period 2. The protocol-
mandated cessation of treatment for patients who lost 50 percent or more of the
improvement gained during period 1 (even if due to a temporary disease fluctuation) may
have contributed to this result. A three-year, open-label extension study that followed the
PIONEER trials suggests long-term efficacy and safety of adalimumab [64]. The study
found a sustained rate of response (achievement of HiSCR) over time among patients who
received 40 mg of adalimumab once weekly for at least 60 weeks.

●Infliximab – A beneficial effect of infliximab for HS was demonstrated in a trial of 38

patients with moderate to severe HS [63]. During the initial randomized, double-blind
phase of the trial, patients were treated with either infliximab infusions (5 mg/kg on weeks
0, 2, and 6) or placebo infusions. This phase was followed by an open-label phase in which
patients in the infliximab group received maintenance doses of infliximab at weeks 14 and
22, and patients in the placebo group were given the opportunity to receive infliximab
according to the same treatment regimen.

By week 8, there was not a significant difference between the treatment and placebo groups
for the primary study outcome (≥50 percent decrease in an unvalidated disease severity
score) [60,63]. However, infliximab therapy was associated with statistically significant
improvements in patient quality of life, pain, and physician global assessment scores. On
physician global assessment, 47 percent of patients in the infliximab group attained 75 to
99 percent improvement compared with none of the placebo-treated patients.
Maintenance therapy may be required to maintain the effects of TNF-alpha inhibitors
[65,66]. In the infliximab trial, three of five patients who were followed through to 52
weeks relapsed after the discontinuation of therapy [63]. In one of the adalimumab trials, all
patients relapsed after the discontinuation of treatment [67]. The transient benefit attained
with treatment with these agents can be useful for reducing disease activity prior to surgery.
Doing so may facilitate definition of the disease margins for the surgeon and may assist
with postsurgical healing [68]. (See "Surgical management of hidradenitis suppurativa",
section on 'Perioperative medication management'.)

Multiple potential adverse effects are associated with anti-TNF therapy, including risks for
infection, heart failure, demyelinating disease, a lupus-like syndrome, and malignancy.
Paradoxical HS occurring during treatment of other disorders with anti-TNF biologic
agents also has been reported [69,70]. The adverse effects of TNF-alpha inhibitors are
discussed separately. (See "Tumor necrosis factor-alpha inhibitors: An overview of adverse
effects".)

Conventional immunosuppressants — Occasionally, systemic glucocorticoids or

cyclosporine are prescribed for HS; however, evidence on the efficacy of these treatments
for HS is limited. In addition, because these drugs may induce severe adverse effects, they
are rarely utilized for long-term therapy [33].

●Systemic glucocorticoids – Based upon clinical experience, a three- to four-day course of

prednisone 40 to 60 mg per day, tapered over the subsequent 7 to 10 days, is often
sufficient for acutely managing inflammation. (See "Major side effects of systemic
glucocorticoids".)

●Cyclosporine – In a retrospective case note review of 18 HS patients with Hurley stage II

or III disease treated with cyclosporine (2 to 3.5 mg/kg per day for 0.5 to 24 months), only
two patients had obvious reduction of symptoms and inflammation evident to both patients
and physicians [71]. Of the remainder, seven patients had "slight improvement," and nine
had "no change." A few case reports describe moderate to marked improvement in
refractory HS with administration of cyclosporine (3 to 5 mg/kg per day orally in two
divided doses) given for several weeks or several months [72-74]. The duration of
cyclosporine treatment is often limited by adverse effects. (See "Pharmacology of
cyclosporine and tacrolimus", section on 'Side effects'.)

Emerging therapies — Ustekinumab, anakinra, and canakinumab are biologic therapies that
may be of benefit for patients with severe and refractory HS based upon limited data. A
case report also describes a potential response of HS to oral tacrolimus.

●Ustekinumab – Positive responses to ustekinumab, an interleukin (IL)-12/23 inhibitor

given via subcutaneous injection, have been reported in patients with moderate to severe
refractory HS [75-77]. An uncontrolled study in which 17 patients with Hurley stage II or
III HS received 45 or 90 mg of ustekinumab at weeks 0, 4, 16, and 28 found at least a 50
percent reduction in the modified Sartorius score in six patients (35 percent) at week 40 and
a 25 to 49 percent reduction in the modified Sartorius score in eight patients (47 percent) at
week 40 [77]. Less improvement occurred in one patient, and two patients had no
improvement. Limitations of this study include the small number of patients and a high
premature dropout rate due to lack of response (three patients), withdrawal of informed
consent (one patient), and an adverse event of urticaria (one patient).

●Anakinra – Anakinra, an antagonist of the IL-1 receptor, may be a treatment option for
HS. A 24-week randomized trial in which 20 adults with Hurley stage II or III HS were
randomly assigned to subcutaneous injections of anakinra (100 mg) or placebo once daily
for 12 weeks found reductions in the disease activity score in six of nine patients (67
percent) in the anakinra group compared with only 2 of 10 patients (20 percent) in the
placebo group at the end of treatment [78]. One patient in the anakinra group was lost to
follow-up. In addition, anakinra therapy was associated with prolongation of the time to
which patients noticed a new exacerbation of HS. Changes in the Sartorius score, patient-
reported disease severity, and quality of life did not differ between the anakinra and
placebo groups. No serious adverse events occurred.

Other reports of the effects of anakinra on HS include a case series and a case report
[79,80]. In the case series of six patients treated with eight weeks of anakinra (100 mg per
day via subcutaneous injection) for moderate to severe HS, the five patients who completed
the treatment course demonstrated reductions in the modified Sartorius score, physician and
patient global assessment scores, and improvement in quality of life [80]. However, the
effects of anakinra diminished upon treatment cessation and rebound of disease was evident
eight weeks after the end of treatment. In the case report, a woman with refractory HS
treated with anakinra (200 mg per day) achieved disease remission within one year with
continued treatment [79].

Failure to respond to anakinra (100 mg per day) has been reported [81-83]. Additional data
are needed to clarify the role of anakinra in HS.

●MABp1 – MABp1, a human monoclonal antibody targeting IL-1 alpha, may be an

effective treatment for moderate to severe HS. In a trial in which 20 patients were randomly
assigned to receive MABp1 or placebo, MABp1 was superior for improving the HiSCR
score after 12 weeks [84].

●Canakinumab – Canakinumab is a human IgG kappa monoclonal antibody that targets the
proinflammatory cytokine IL-1 beta. In a case report, two patients with severe HS had
improvement in both Sartorius scores and pain scores during canakinumab therapy [85].

●Tacrolimus – Clearance of HS after a change in immunosuppression regimen from

cyclosporine to oral tacrolimus is documented in a case report [86]. The patient was a renal
transplant recipient who developed HS two years after renal transplantation.

Surgery — Surgical procedures may be performed on individual nodules or sinus tracts and
in severe cases may be used to excise an entire affected area [56]. Surgery should not be
used in isolation; combining surgery with medical therapy provides the best chance for
preventing the development of new lesions and controlling disease progress.
The surgical approach generally becomes more aggressive with higher-stage disease. (See
"Surgical management of hidradenitis suppurativa", section on 'General considerations' and
'Hurley stage I (mild disease)' above and 'Hurley stage II' above and 'Hurley stage III
(severe and refractory disease)' above.)

Surgical procedures for HS are discussed in detail separately. (See "Surgical management
of hidradenitis suppurativa", section on 'Local procedures' and "Surgical management of
hidradenitis suppurativa", section on 'Wide excision and reconstruction'.)

Other nonsurgical therapies — Additional therapies that have been reported to be effective
in small numbers of patients are reviewed below. In particular, metformin, oral zinc, and
laser or light therapies may be useful as adjunctive or alternative therapies.

Metformin — Early evidence suggests that metformin may be a useful treatment for HS
[22,23]. Support for the efficacy of metformin for HS stems from a 24-week prospective
study of 25 nondiabetic patients with HS who were treated with metformin (initial dose 500
mg per day, maximum dose 500 mg three times per day), almost all of whom had failed to
achieve sufficient responses to other therapies [23]. Eighteen patients achieved clinical
improvement, including seven who achieved at least a 50 percent reduction in Sartorius
score. Insulin and insulin-like growth factor may contribute to HS, providing a potential
underlying mechanism for a beneficial effect of metformin in HS, along with possible
weight loss. Additional studies will be useful for confirming the efficacy of metformin in
HS. The development of nausea can be a limiting factor for treatment with metformin.

Zinc supplementation — Zinc salts have anti-inflammatory and antiandrogenic properties,

and there are limited data for possible benefit in HS [87]. The efficacy of zinc gluconate
(90 mg per day) was investigated in a pilot study of 22 patients who had failed to achieve
satisfactory improvement with systemic antibiotic therapy, antiandrogens, isotretinoin, or
surgery [87]. All but one patient had Hurley stage I or II disease. Among the patients, eight
(36 percent) achieved complete responses (disappearance of lesions or no new lesions for
≥6 months), and the remainder achieved partial remissions (≥50 percent reduction in the
number of nodules and/or a shorter duration of inflammatory lesions). However, relapses
occurred upon tapering of the dose to ≤60 mg per day.

Additional support for a beneficial effect of zinc gluconate in HS stems from a prospective
study of 12 patients with Hurley stage I or II HS [88]. Deficiencies in innate immune
markers detected in lesional and nonlesional skin from patients with HS were improved
following three months of treatment with zinc gluconate (90 mg per day).

Gastrointestinal upset may occur as a consequence of zinc sulfate therapy [87]; zinc
gluconate (30 mg twice or three times per day in adults) may be better tolerated. Of note,
zinc may displace copper, reducing copper absorption.

Laser and light therapy — In a randomized within-participant trial of 22 patients with

Hurley stage II or III disease, treatment of the affected skin region with a 1064 nm
neodymium-doped yttrium aluminum garnet (Nd:YAG) laser significantly reduced disease
severity in inguinal, axillary, and inframammary sites [89]. The mechanism of action of the
Nd:YAG laser in HS may involve follicular destruction or dermal heating leading to the
disruption of the inflammatory infiltrate [90].

Treatment with intense pulsed light of affected skin regions (axilla, groin, or inframammary
area) was also associated with improvement in disease severity in a randomized within-
participant trial of 18 patients with Hurley stage II or III HS [91].

Photodynamic therapy combines use of a photosensitizer and a light source to induce

cellular destruction through porphyrin activation. Treatment of areas of involvement of HS
with photodynamic therapy using a topical photosensitizer has yielded variable results [92-
96]. An intralesional technique for photodynamic therapy was associated with improvement
in case reports and a small retrospective study [97,98]. Intralesional photodynamic therapy
is rarely used clinically.

External beam radiation — A retrospective study of 231 patients with refractory HS treated
with radiotherapy found that 38 percent had complete resolution of symptoms and an
additional 40 percent had some degree of improvement in symptoms following radiation
treatment [99]. Although radiation therapy administered to the affected skin region may be
effective [99,100], this modality is rarely used for HS due to concern for the occurrence of
long-term adverse effects (eg, chronic radiation dermatitis, ulceration, cutaneous
malignancy) [16]. (See "Radiation dermatitis" and "Clinical manifestations, prevention, and
treatment of radiation-induced fibrosis".)

Fumarates — Fumarates are anti-inflammatory and immunomodulatory agents used in the

treatment of psoriasis. In an uncontrolled pilot study in which seven patients with
treatment-refractory moderate to severe HS were treated with oral fumarates, three patients
seemed to improve during therapy and four patients discontinued treatment because of lack
of efficacy [101]. Data are insufficient to recommend routine use of this therapy.

Vitamin D3 — Vitamin D3 supplementation has been proposed as a treatment for HS based

upon hypotheses that a deficiency in innate immunity may contribute to HS and that
vitamin D3 may play an important role in innate immunity. In an uncontrolled study in
which 14 patients with HS and vitamin D3 deficiency (defined as 25-hydroxyvitamin D3
level <30 ng/mL) received vitamin D3 supplementation (100,000 to 600,000 international
units given at baseline and/or after three months depending on the serum vitamin D level),
11 (79 percent) had at least a 20 percent reduction in the number of nodules after six
months [102]. Additional study is necessary to confirm efficacy of vitamin D3
supplementation in HS.

Other — Treatments that have been reported as beneficial in case reports include botulinum
toxin injections [103,104], cryoinsufflation [105], and the glucagon-like peptide-1 agonist
liraglutide [106]. Further study will be useful for confirming the efficacy of these therapies.

Microwave ablation, a noninvasive procedure that destroys sweat glands and hair follicles
through thermolysis, might not be effective for HS. A randomized trial in which outcomes
of a single treatment in one axilla were compared with findings in the untreated
contralateral axilla was discontinued after interm analysis of nine patients suggested no
benefit and potential harm of this intervention [107]. Of the eight patients who completed
treatment, five experienced worsening of disease after treatment.

PROGNOSIS — Early diagnosis of HS is essential because most cases can be effectively

treated when diagnosed at an early stage [5]. Stage III disease is very difficult to manage
and requires a multidisciplinary treatment approach, with coordination between
dermatologists, dermatology specialist nurses, surgeons who have axillary and inguinal
expertise, wound healing experts, and input from clinical psychologists. Although cure is
possible in a skin region after extensive surgery [108], this does not prevent disease
progression in other affected skin regions, and so further medical treatment is required.

Rarely, squamous cell carcinoma develops within sites of HS. Squamous cell carcinoma
tends to be seen in patients who have suffered from HS for 10 years or more and is often
advanced at diagnosis [109-112].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines

from selected countries and regions around the world are provided separately. (See "Society
guideline links: Hidradenitis suppurativa".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education

materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are
written in plain language, at the 5th to 6th grade reading level, and they answer the four or
five key questions a patient might have about a given condition. These articles are best for
patients who want a general overview and who prefer short, easy-to-read materials. Beyond
the Basics patient education pieces are longer, more sophisticated, and more detailed. These
articles are written at the 10th to 12th grade reading level and are best for patients who want
in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to
print or e-mail these topics to your patients. (You can also locate patient education articles
on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Hidradenitis suppurativa (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Hidradenitis suppurativa (HS) is a chronic, painful, inflammatory skin disorder of the

folliculopilosebaceous units characterized by the development of inflammatory nodules,
pustules, sinus tracts, and scars, primarily in intertriginous areas. (See "Hidradenitis
suppurativa: Pathogenesis, clinical features, and diagnosis".)

●Physical pain, odor, chronic drainage, and disfigurement are common features of this
disorder. The symptoms of HS often have a profound negative effect on quality of life. (See
"Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis".)
●Successful treatment of HS requires prevention of new lesions in all stages of the disease
to limit disease progression. Treatment modalities include patient self-management and
medical and surgical interventions. (See 'Treatment' above and "Surgical management of
hidradenitis suppurativa".)

●Patient self-management strategies are important and include avoidance of skin trauma,
smoking cessation, and weight management where relevant. Patient education and
psychologic support are additional important components of patient management. (See
'General approaches' above.)

●The level of disease severity strongly influences the approach to the treatment of HS.

•Patients with Hurley stage I HS present with single or multiple nodules and abscesses
without associated sinus tracts or scarring. For these patients, we suggest daily treatment of
the involved areas with topical clindamycin (Grade 2B). A topical antiseptic wash can also
be used for showering.

Acutely inflamed lesions may require analgesia, such as nonsteroidal anti-inflammatories.

Punch debridement (partial deroofing [unroofing]) or intralesional corticosteroid injection
may be performed to reduce inflammation. Topical resorcinol is an alternative patient-
administered treatment. (See 'Hurley stage I (mild disease)' above and "Surgical
management of hidradenitis suppurativa", section on 'General considerations' and "Surgical
management of hidradenitis suppurativa", section on 'Punch debridement'.)

•Patients with Hurley stage II HS exhibit recurrent inflamed nodules and abscesses, some
of which lead to sinus tracts and scarring. Treatment includes continuation of preventive,
plus medical and surgical, therapy.

For the medical treatment of the inflammatory component of Hurley stage II disease, we
suggest oral tetracyclines for first-line therapy (Grade 2C). Treatment with doxycycline
(100 mg once daily or twice daily) is common and should be continued for several months.
Combination therapy with clindamycin and rifampin can be helpful for suppurative disease
unresponsive to oral tetracyclines. Acitretin and antiandrogenic agents may also provide
benefit. (See 'Hurley stage II' above.)

The sinus tracts of Hurley stage II HS will not resolve with antibiotic therapy and may
require surgical deroofing (unroofing) if they are recurrently inflamed. (See 'Hurley stage
II' above and "Surgical management of hidradenitis suppurativa", section on 'General
considerations' and "Surgical management of hidradenitis suppurativa", section on
'Unroofing (local or extensive)'.)

•Hurley stage III HS is characterized by diffuse or nearly diffuse involvement of the

affected area (intertriginous or not) with fluctuant nodules, interconnecting sinus tracts, and
extensive scarring. For the nonsurgical treatment of Hurley stage III HS that cannot be
managed with oral antibiotics, oral retinoids, or hormonal therapy, we recommend
adalimumab as the next-line therapy (Grade 1A).
•For patients with severe HS in a localized area that has failed to respond sufficiently to
medical therapy and limited surgical therapy (eg, deroofing), extensive surgical excision
offers the best chance to provide permanent relief. (See 'Hurley stage III (severe and
refractory disease)' above and "Surgical management of hidradenitis suppurativa", section
on 'General considerations' and "Surgical management of hidradenitis suppurativa", section
on 'Wide excision and reconstruction'.)

ACKNOWLEDGMENTS

The editorial staff at UpToDate would like to acknowledge Lynette Margesson, MD,
FRCPC, FAAD, who contributed to an earlier version of this topic review.

We are saddened by the death of F. William Danby, MD, FRCPC, FAAD, who passed
away in November 2016. UpToDate wishes to acknowledge Dr. Danby's past work as an
author for this topic.

Use of UpToDate is subject to the Subscription and License Agreement.

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81. van der Zee HH, Prens EP. Failure of anti-interleukin-1 therapy in severe
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83. Russo V, Alikhan A. Failure of Anakinra in a Case of Severe Hidradenitis
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Dermatol Surg 2017; 43:107.

Topic 7605 Version 42.0

Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis
Author:
John R Ingram, MD, PhD
Section Editors:
Robert P Dellavalle, MD, PhD, MSPH
Mark V Dahl, MD
Deputy Editor:
Abena O Ofori, MD

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is
complete.
Literature review current through: Oct 2018. | This topic last updated: Apr 30, 2018.

INTRODUCTION — Hidradenitis suppurativa (HS, from the Greek hidros = sweat and
aden = glands) is a chronic inflammatory skin condition that is also known as acne inversa
and, historically, as Verneuil's disease [1,2]. Although the name "hidradenitis suppurativa"
implies a suppurative disorder that primarily involves sweat glands, increasing knowledge
of the pathogenesis of the condition has led to the prevailing theory that HS is a chronic
follicular occlusive disease involving the follicular portion of folliculopilosebaceous units
(FPSUs).

The primary sites of involvement for HS are the intertriginous skin areas of the axillary,
groin, perianal, perineal, and inframammary regions, though HS can occur in any skin area
that contains FPSUs. The clinical manifestations vary, ranging from recurrent inflamed
nodules and abscesses to draining sinus tracts and bands of severe scar formation. The
associated pain, malodor, drainage, and disfigurement that accompany HS contribute to a
profound psychosocial impact of the disease on many patients [3].

Early HS lesions often mimic other disorders; misdiagnosis of HS as recurrent furunculosis

or "boils" is common. Prompt recognition of the correct diagnosis is important. An early
and accurate diagnosis facilitates the initiation of a treatment plan aimed at minimizing the
risk of progression to disabling, end-stage disease.

The pathogenesis, clinical manifestations, and diagnosis of HS will be discussed here.

Treatment is reviewed separately. (See "Hidradenitis suppurativa: Treatment".)

EPIDEMIOLOGY — Estimates of the prevalence of HS have varied, ranging from less

than 1 percent to 4 percent [4-9]. The first population-based incidence study of HS found an
annual age- and sex-adjusted incidence of 6 per 100,000 in a county in Minnesota [10]. A
subsequent, larger, population-based study that utilized data from a database of more than
48 million patients in the United States found a higher incidence (11.4 per 100,000
population) [11].

The onset of symptoms typically occurs between puberty and age 40, with usual onset in
the second or third decade of life. Women are more likely to develop HS than men [11,12].
In a French series of 618 consecutive patients with HS, the sex ratio was 3.6:1 [12]. In
addition, the large population-based study in the United States found that the incidence of
HS was twice as high among women than among men; the highest age-specific incidence
was among patients between the ages of 18 and 29 years [11].

PATHOGENESIS — The pathogenesis of HS is not fully understood; however, new

evidence is emerging [13]. As opposed to early theories that implicated apocrine glands as
the primary contributors to HS, most authors now support follicle-centered theories for the
pathogenesis of HS. Follicular occlusion, follicular rupture, and an associated immune
response appear to be important events in the development of the clinical manifestations of
HS. The basic principles that underlie follicle-centered theories are reviewed below.

Mechanism — Follicular occlusion is the most likely event responsible for the initial
development of HS lesions. Follicular occlusion appears to result from ductal keratinocyte
proliferation (follicular epithelial hyperplasia), causing follicular hyperkeratosis and
plugging [14]. Proposed contributors have included the effects of hormones and nicotine on
the follicular epithelium [14-16] (see 'Associated factors' below). It may be that that anoxia
within the follicular duct secondary to follicular epithelial hyperplasia contributes to the
disruption of normal terminal differentiation of follicular keratinocytes, leading to follicular
plugging [17].

As a consequence of the failure of terminal differentiation, the keratinocytes do not separate

and the follicular duct expands. Mechanical stress (pressure, friction, or shear) on skin,
particularly in intertriginous areas, results in leakage of molecular-sized antigens that
stimulate the adaptive immune system as well as stimuli for the innate immune system.
Subsequent cytokine release leads to activation of keratinocytes that release their own
proinflammatory mediators [18]. Perifolliculitis is detected at this stage [14,19]. If
sufficient repair of the compromised follicular duct does not occur, the follicular duct
eventually may rupture, leading to the release of macro follicular contents (eg, keratin
fragments derived from corneocytes and hair, sebum products, bacteria), and the further
recruitment of inflammatory cells [18]. Over time, the initial acute inflammatory response
may evolve into chronic foreign body-type granulomatous inflammation.

Defects in the support of the follicular wall may predispose individuals with HS to
follicular rupture. A study that compared skin specimens from patients with HS to
specimens from normal controls found that axillary specimens from patients with HS
demonstrated a marked reduction in periodic acid-Schiff (PAS) staining of the basement
membrane zone at the junction between the sebaceous gland and the follicle (sebofollicular
junction) [20]. In contrast, continuous PAS staining was noted along the basement
membrane zone of the folliculopilosebaceous unit in specimens from patients in the control
group.

Follicular rupture may promote the formation of sinus tracts through the release of stem
cells from the bulge area of the hair follicle that subsequently proliferate and form epithelial
strands [18,20,21]. Formed sinus tracts eventually open onto the skin surface and become
chronically inflamed.
Questions remain about the role of the immune system in HS [22], particularly whether
dysregulation of the immune system is a contributor. Although activation of the innate and
adaptive immune systems is an expected response to the release of antigens and other
proinflammatory stimuli from a ruptured follicular duct, a role for immune system
dysregulation in HS is suggested by similarities between HS and Crohn disease, an
inflammatory bowel disorder hypothesized to involve dysregulation of the innate and
adaptive immune systems. HS and Crohn disease share histologic features, and there is a
well-established epidemiologic association between the two conditions [23,24]. Moreover,
the inflammatory processes of both diseases may respond to anti-tumor necrosis factor
(TNF)-alpha therapies [13,18,25-27].

Further studies are necessary to explore whether immune dysregulation is a primary

contributor to the initiation of HS. Interleukin (IL)-17 and the caspase-1-associated
cytokines IL-1beta and IL-18 may be involved in the disease pathogenesis [28]. Increased
understanding of the role of the immune system may aid in the discovery of new
treatments.

Associated factors — Genetic susceptibility, mechanical stresses on the skin, obesity,

smoking, diet, and hormonal factors are repeatedly cited as factors that may be associated
with the development or exacerbation of HS. The relationships between these factors and
HS are discussed below:

●Genetics – Genetic susceptibility appears to be an important contributor to HS. It is

estimated that approximately 40 percent of patients with HS have an affected first-degree
family member [29]. Compared with other HS patients, patients with early-onset HS (onset
prior to age 13) may be more likely to have a family history of the disease [30]

Familial occurrences of HS resembling an autosomal dominant-like inheritance pattern

have been documented (MIM #142690). A candidate locus for HS at chromosome 1p21.1-
1q25.3 was identified via a genome-wide scan in a Chinese family that demonstrated an
autosomal dominant pattern of inheritance and a particularly widespread form of HS [31].
However, this locus was not associated with HS in two additional families with a more
classic presentation in which an autosomal dominant inheritance pattern appeared to be
present [32].

Further studies are necessary to clarify the impact of genetics on risk for HS. The finding of
mutations in genes encoding the components of gamma-secretase, an intramembranous
protease complex that cleaves multiple transmembrane proteins, in kindreds with HS may
contribute to further understanding of the disease [33-36]. Gamma-secretase cleaves the
intracellular domain of Notch, a transmembrane receptor protein that plays an important
role in keratinocyte differentiation. Although it appears that germline gamma-secretase
mutations may contribute to HS in only a small subset of patients [33], familial and mouse
studies support the theory that inherited or acquired impairment of Notch signalling may
play a key role in disease development [37]. The gamma-secretase mutations that have been
reported in patients with HS include mutations in genes for presenilin-1 (PSEN1),
presenilin enhancer-2 (PSENEN), and nicastrin (NCSTN) [34,38-41]. TNF gene
polymorphisms also may play a role in susceptibility to HS [42].
●Mechanical stress – Increased mechanical stress (pressure, friction, shear) on
intertriginous skin and other areas (eg, beltlines, brassiere straps, and other sites of clothing
friction) likely contributes to the localization of HS. Mechanical stress on skin has been
postulated to contribute to HS via increasing the chance of follicular occlusion and
follicular rupture [4,18].

●Obesity – Excess weight is more common in patients with HS than in the general
population; however, the disease is not limited to overweight or obese individuals. In a
case-control study of 302 patients with HS and 906 controls, a body mass index (BMI) ≥30
was present in 21 percent of patients with HS versus only 9 percent of controls and a BMI
between 25 and 29 was present in 22 percent of HS patients versus 17 percent of controls
[43]. One theory for the association between excess weight and HS is that dietary choices
that increase risk for the disease are those that also contribute to obesity [44] (see
"Hormones" below). Several studies have found a positive correlation between increasing
BMI and disease severity [45-50].

As in acne vulgaris, hormonal changes associated with obesity may result in relative
androgen excess and are proposed to increase follicular plugging [51]. Moreover, the
comparatively larger size of intertriginous areas, local skin irritation from sweat retention,
narrowed follicular orifices secondary to intrafollicular keratin hydration during skin
occlusion, and obesity-related increases in levels of circulating proinflammatory cytokines
may contribute [4,18,52]; however, these factors are also present in obese individuals
without HS.

●Smoking – There is a strong relationship between smoking and HS; the majority of
affected patients are smokers [10,43,45,50,53]. An American retrospective cohort study
that included approximately four million tobacco smokers and approximately eight million
nonsmokers found the overall incidence of HS higher among tobacco smokers (0.2 versus
0.1 percent) [54]. In a French case-control study of 302 clinically assessed patients with HS
and 906 controls, 76 percent of the patients with HS versus 25 percent of the controls were
current smokers [43]. This particular study did not find an association between smoking
and severity of disease; however, other studies have found more severe disease in smokers
than nonsmokers [10,45]. In a study in which the modified Hidradenitis Suppurativa Score
was used to assess disease severity in 115 patients with HS, the median score was
significantly higher among the smokers than among the nonsmokers [45]. Stimulatory
effects of nicotine and other components of tobacco on follicular occlusion, neutrophil
chemotaxis, TNF-alpha production by keratinocytes, and Th17 cells have been cited as
potential contributing factors [13,55].

●Hormones – An impact of hormones on HS is suggested by observations of the rarity of

HS in prepubertal children and improvement in HS during treatment with antiandrogenic
agents [56-59]. In addition, some women experience perimenstrual flares, and in this
subgroup, amelioration of disease severity during pregnancy was found in a retrospective
survey [60]. Hyperandrogenemia seems unlikely to be a contributor to HS based upon a
series of 66 women with HS that found no evidence of biochemical hyperandrogenism
when compared with controls matched for age, weight, and hirsutism [61].
Additional studies are necessary to determine the role of hormones in HS.

●Bacteria – The role of bacteria in HS is controversial. Cultures from early, unruptured HS

lesions are usually sterile. Older and ruptured lesions and sinuses may demonstrate a wide
variety of bacteria (eg, staphylococci, streptococci, Gram-negative rods, and anaerobic
bacteria). Coagulase-negative staphylococci are frequently present [62,63]. Positive
cultures may represent contaminants from normal skin flora or secondary infection. One
theory suggests that bacteria may contribute to HS by promoting an inflammatory response
[26], and a role for a bacterial biofilm in the persistence of the inflammatory process of HS
has been proposed [64,65]. In addition, a case-control study found significant differences in
the microbiome of lesional and nonlesional skin in 30 patients with HS compared with 24
healthy controls [66].

●Drugs – In support of a contribution of androgens to HS, there are reports of female

patients in whom treatment with oral contraceptives containing androgenic progestins,
intramuscular medroxyprogesterone acetate, or levonorgestrel in an intrauterine device may
precipitate or worsen HS [67]. Lithium therapy has been linked to HS in a small number of
patients [68,69]. Paradoxically, HS has been reported as an adverse effect of anti-TNF-
alpha therapies and other biologic treatments given for other chronic inflammatory diseases
[70].

CLINICAL MANIFESTATIONS — HS predominantly occurs in intertriginous areas. HS

can affect the axillae (most common site), inguinal area, inner thighs, perianal and perineal
areas, mammary and inframammary regions, buttocks, pubic region, scrotum, vulva, trunk,
and, occasionally, the scalp and retroauricular areas (picture 1A-C) [71]. Early-onset HS
may be associated with a greater risk for widespread disease [30].

Sex influences the distribution of HS. Primary sites of involvement in women are the groin
or upper inner thigh, axilla, chest (including breast and inframammary regions), and the
buttocks or gluteal clefts [10,72]. In men, primary sites of involvement are the groin or
thigh, axilla, perineal or perianal regions, and buttocks or gluteal cleft [10,48,72]. Both
sexes regularly show involvement in non-intertriginous skin, particularly at sites of skin
compression and friction. Beltlines, waistbands, abdominal folds, and brassiere straps or
bands are common locations.

Given the heterogeneity in clinical presentation within the HS phenotype, attempts have
been made to generate disease subclassifications [12]. However, these models have not
been related to response to treatment.

The primary visible lesions of HS are inflammatory nodules. Sinus formation, clusters of
open comedones (tombstone comedones), and scarring are the result of recurrent or
persistent disease.

●Inflammatory nodules – Most frequently, the first lesion is a solitary, painful, deep-
seated inflamed nodule (0.5 to 2 cm in diameter), often in an intertriginous area. Onset can
be insidious, with occasional solitary, painful nodules that can last for several days to
months. Recurrent episodes of nodule formation may occur in the exact same location or in
the same skin region.

The diagnosis of HS is frequently missed at this stage. In a survey including 517 patients in
24 countries, diagnostic delay on average was seven years [73]. The inflammatory nodules
are commonly misdiagnosed as "boils" or furunculosis. Unlike furuncles, primary HS
lesions are deep-seated and round-topped, and lack the pointed appearance of furuncles [5].
The nodules are painful, and depending on the location and size of lesions, patients may be
unable to walk or sit without pain [74].

After a variable period of time, the nodule often progresses to form an abscess that may
open to the skin surface spontaneously (or as a result of manipulation by the patient),
yielding purulent or serosanguineous drainage (picture 2). Pain often improves after
drainage. Other nodules regress without draining, usually within one to several weeks [29].
In a questionnaire-based study of 110 patients with HS, patients reported that the average
duration of a single painful lesion was seven days, although approximately 60 percent of
patients described at least one persistent painful nodule that failed to subside completely
[29]. The number and frequency of nodules varies widely among patients. Patients in this
study reported a median of two nodules per month (range 1 per year to 30 per month).

●Sinus tracts – Sinus tracts are typical findings in HS that persist for months or years and
contribute to symptomatology (picture 3). The development of multiple recurrent nodules
within a limited area may predispose to the formation of intercommunicating sinuses.
Patients with sinus tracts often experience intermittent release of blood-stained,
seropurulent, malodorous discharge. Sinus tracts are not always palpable, and in some
cases, a sinus tract becomes apparent only when a substance (eg, local anesthetic) injected
into a lesion appears at a site distant from the site of injection. Ulceration may accompany
sinus tract formation. Occasionally, pyogenic granulomas develop at sinus tract openings
[75]. (See "Pyogenic granuloma (Lobular capillary hemangioma)".)

●Comedones – Open comedones often appear in long-standing HS, commonly as double-

headed or multi-headed open comedones [76]. These structures are often described as
"tombstone comedones" because they reflect end stage damage to the
folliculopilosebaceous unit with associated loss of the sebaceous gland and hair [77].

Closed comedones may also develop in HS. The closed comedones of HS are essentially
tiny cysts that result from continued keratin production by the follicular epithelium lining
the residual stub of the follicle above the destroyed sebofollicular junction. Closed
comedones are not primary lesions and therefore, are not present in early cases of HS. The
presence of comedones is not required for the diagnosis of early disease.

●Scarring – The appearance of healed areas ranges from individual, pitted, acneiform scars
after resolution of small nodules to dense, fibrotic bands or indurated, thick, scarred plaques
affecting the whole affected area. Scars may also be atrophic (particularly on the trunk) or
keloidal, and scarring on the buttocks sometimes manifests as multiple pitted scars. In
patients with active disease, scarring is accompanied by inflammatory nodules and draining
sinuses.
In areas of lax flexural skin, such as the axilla, scarring can result in thick, linear, rope-like
bands. Severe scarring in the axilla may result in reduced mobility of the arm or lymphatic
obstruction leading to lymphedema. Groin involvement may lead to lymphedema of the
pubis or the entire vulvar area in women or penile and/or scrotal lymphedema in men [78].

HS has a significant impact on patient quality of life [3]. Pain is a major cause of disutility,
along with scarring, the unpredictable discharge, the necessary dressings, and the
accompanying odor, which are embarrassing and humiliating [79]. As a result, patients may
experience social isolation, depression, failed relationships, and loss of (or failure to gain)
employment [80,81]. An increased suicide risk has also been reported among patients with
HS [82]. Prodromal symptoms may also be experienced prior to disease flares, including
fatigue and localized pruritus or paresthesias [83].

CLINICAL STAGING — The Hurley clinical staging system frequently is used to divide
patients with HS into three disease severity groups [84].

●Stage I – Abscess formation (single or multiple) without sinus tracts and

cicatrization/scarring (picture 4)

●Stage II – Recurrent abscesses with sinus tracts and scarring, single or multiple widely
separated lesions (picture 5)

●Stage III – Diffuse or almost diffuse involvement, or multiple interconnected sinus tracts
and abscesses across the entire area (picture 6)

The majority of patients with HS exhibit stage I disease. In a French series of 302
consecutive patients with HS, stage I, II, and III disease were observed in 68, 28, and 4
percent of patients, respectively [46].

ASSOCIATED DISORDERS AND SYNDROMES — A variety of disorders have been

linked to HS.

●Emerging data indicate that patients with HS may have increased incidence of (and risk
for) metabolic syndrome and related comorbidities, a constellation of metabolic and
secondary disorders including diabetes, obesity, insulin resistance, dyslipidemia,
hyperglycemia, and hypertension [49,85-90]. In combination with higher rates of smoking
in HS, these factors contribute to a higher risk of cardiovascular-associated death; the risk
was nearly double that of a control group in Danish HS patients [91]. In this climate,
patients with HS should be offered the benefit of a multidisciplinary approach to aid in
prevention, detection, and management of cardiovascular disease. (See "The metabolic
syndrome (insulin resistance syndrome or syndrome X)".)

●Several studies suggest an association between HS and inflammatory bowel disease (IBD)
[24,26,92-94]. As an example, an Israeli cross-sectional study of 3207 patients with a
diagnosis of HS and 6412 matched controls found an association with Crohn disease (odds
ratio 2.03, 95% CI 1.14-3.62) [92]. In addition, a population-based cohort study of 679
patients with Crohn disease or ulcerative colitis in the United States found these patients
more likely to develop HS than the general population (incidence rate ratio 8.9, 95% CI
3.6-17.5) [93]. Patients with IBD and HS tend to be younger than patients with IBD alone
and are more likely to be smokers and have other comorbid conditions (eg, obesity,
diabetes mellitus) [95,96]. (See "Clinical manifestations, diagnosis and prognosis of Crohn
disease in adults" and "Clinical manifestations, diagnosis, and prognosis of ulcerative
colitis in adults".)

●Acne vulgaris is a common condition that may be seen in association with HS. Acne
vulgaris can be particularly severe and difficult to treat in patients with HS [4]. (See
"Pathogenesis, clinical manifestations, and diagnosis of acne vulgaris".)

●HS is a component of the follicular occlusion tetrad. The follicular occlusion tetrad
consists of four diagnoses: HS, acne conglobata, dissecting cellulitis of the scalp, and
pilonidal sinus. These four conditions are considered to share follicular occlusion as a key
pathogenic event and may appear in conjunction with one another in individual patients
[94,97]. (See "Pathogenesis, clinical manifestations, and diagnosis of acne vulgaris",
section on 'Acne conglobata' and "Evaluation and diagnosis of hair loss", section on
'Cicatricial alopecia' and "Pilonidal disease".)

●There are isolated reports of patients in whom HS has appeared to develop within a
syndrome-like presentation consisting of several inflammatory diseases. PAPASH
syndrome (pyogenic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis)
has been described in an adolescent in whom a mutation in the PSTPIP1 gene was detected
[98]. The term PASH syndrome has been used to refer to patients with pyoderma
gangrenosum, acne, and suppurative hidradenitis [99-101]. Genetic analysis has revealed a
PSTPIP1 mutation in a patient with PASH syndrome [102]; however, in two other patients
with PASH syndrome PSTPIP1 mutations were absent [99].

Other conditions, including inflammatory, neoplastic, and genetic disorders have been
associated with HS [103]. A table listing examples of associated disorders is provided
(table 1).

HISTOPATHOLOGY — The histopathologic findings in HS vary according to the stage of

the lesion (picture 7). Common early features include follicular hyperkeratosis, follicular
plugging, follicular dilation, and lymphocytic perifolliculitis [14]. Biopsies of established
areas of disease can demonstrate additional features, including psoriasiform hyperplasia of
the interfollicular epithelium [14] or a dense, mixed inflammatory infiltrate involving the
lower half of the dermis and subcutis [104]. In addition, chronic abscesses, sinus tracts
lined by stratified squamous epithelium, and granulation tissue with or without foreign
body giant cells may be present. Destruction of folliculopilosebaceous units, fibrosis, and
incidental peri-apocrine and peri-eccrine inflammation also may be evident [52,75].

DIAGNOSIS — The diagnosis of HS is made from the patient history and recognition of
characteristic clinical manifestations [5].
History and physical examination — The three main clinical features that support a
diagnosis of HS are as follows:

●Typical lesions (multiple deep-seated inflamed nodules, tombstone comedones, sinus

tracts, abscesses and/or fibrotic scars)

●Typical locations (in particular, axillae, groin, inframammary areas; often bilateral
distribution)

●Relapses and chronicity

A diagnosis of HS is straightforward in patients who demonstrate the constellation of

recurrent inflammatory nodules, sinus tracts, and hypertrophic scarring in intertriginous
areas. However, the location, type, number, and severity of typical lesions vary widely
among patients with HS. Thus, clinicians should maintain high suspicion for the diagnosis
for all patients who present with recurrent inflammatory nodules, particularly when
intertriginous involvement is present. Recognition of this feature and the characteristic
patterns of scarring may allow for recognition of the disease at early stages.

The patient history can be a valuable tool in the diagnosis of HS. Onset in adolescence or
young adulthood, a history of recurrent or persistent disease, or a family history of HS offer
support for a diagnosis. Patients with atypical features also should be questioned about
gastrointestinal symptoms to identify patients who may benefit from an evaluation for
Crohn disease. (See 'Differential diagnosis' below and "Clinical manifestations, diagnosis
and prognosis of Crohn disease in adults".)

A full skin examination should be performed in patients with suspected HS. This allows for
an assessment of the extent and severity of involvement and the recognition of features that
are more or less consistent with this disease.

Laboratory studies — A skin biopsy usually is not necessary for diagnosis. However, in
cases in which the diagnosis is uncertain, a biopsy can be useful for excluding other
disorders. A biopsy should also be performed if features suggestive of squamous cell
carcinoma (SCC) are present. SCC may appear as a slowly growing, firm, nonfluctuant and
nondraining mass that is superficially eroded or ulcerated and is located in an area of
chronic HS.

Routine bacterial cultures are not indicated [5]. Cultures are reserved for cases in which the
clinical findings are suggestive of a primary infectious disorder rather than HS or if there is
clinical evidence of secondary cellulitis [5]. No serologic studies confirm a diagnosis of
HS.

Imaging — Radiologic imaging is not necessary for the diagnosis of HS. However,
ultrasound may be useful for preoperative assessment of the subclinical extent of disease
[5,105-107]. Magnetic resonance imaging may be helpful for delineating extensive
anogenital disease [108].
DIFFERENTIAL DIAGNOSIS — A few diseases produce inflamed nodules, recurrent
abscesses, or sinus tracts that may be mistaken for HS. The patient history and clinical
findings are usually sufficient for distinguishing HS from other diseases.

●Follicular pyodermas (folliculitis, furuncles, carbuncles) – Folliculitis, furuncles

("boils"), and carbuncles are infections arising from hair follicles. Infectious folliculitis
usually presents with multiple, superficial, inflammatory papules, each surrounding a hair
follicle, with or without an overlying pustule. Infection that progresses to a nodular abscess
is termed a furuncle. Carbuncles occur when several inflamed follicles converge into a
single inflammatory mass with purulent drainage emanating from multiple areas. Unlike
HS, which typically exhibits a chronic and recurrent course, follicular pyodermas are
transient lesions that usually respond rapidly to appropriate antibiotic therapy. In addition,
the follicular pyodermas do not cause the comedones, persistent sinus tracts, and
hypertrophic scarring observed in HS. (See "Cellulitis and skin abscess: Clinical
manifestations and diagnosis".)

●Acne vulgaris – Like HS, acne vulgaris demonstrates follicular hyperkeratinization

leading to comedones, inflammatory nodules, and scarring. Although there is some overlap
in the distribution of the two diseases, acne vulgaris primarily occurs on the face, upper
chest, and back, whereas HS primarily involves the axillae, groin, buttocks, and
inframammary folds. In addition, HS typically causes more deep-seated lesions, and several
of its consequences, including sinus tracts and "rope-like" scars, provide characteristic
clinical features. (See "Pathogenesis, clinical manifestations, and diagnosis of acne
vulgaris".)

●Intergluteal pilonidal disease – Intergluteal pilonidal disease manifests as a cavity, pit,

or sinus in the area of the natal cleft that may become inflamed, swollen, and painful with
associated mucoid, purulent, or bloody drainage. Along with hidradenitis suppurativa, acne
conglobata, and dissecting cellulitis of the scalp, pilonidal disease is a member of the
follicular occlusion tetrad. Thus, pilonidal disease and HS may occur in the same patient,
and there is ongoing debate regarding whether pilonidal disease is a phenotypic variant of
HS [109]. (See "Pilonidal disease".)

●Crohn disease – Perianal and vulvar manifestations of Crohn disease include abscesses,
rectoperineal and rectovaginal fistulae, sinus tracts, fenestrations, and scarring, as well as
classic "knife-cut" ulcers in the inguinal, genitocrural, or interlabial folds (picture 8A-B). A
history of gastrointestinal Crohn disease supports this diagnosis; however, the simultaneous
development of Crohn disease and HS has been reported [25,26]. (See "Perianal
complications of Crohn disease".)

●Granuloma inguinale – Granuloma inguinale (donovanosis) is a sexually transmitted

infection caused by Klebsiella granulomatis that usually occurs on the vulva, penis,
scrotum, glans, inguinal folds, or perianal skin (picture 9) [110]. Lesions usually present as
succulent red ulcers with granulation tissue that bleeds easily and with a foul odor. The
presence of an enlarging ulcer with an undermined border suggests this diagnosis over HS.
The causative organism cannot be cultured. Diagnosis requires visualization of Donovan
bodies on tissue crush preparation or biopsy.
Examples of other disorders that may enter the differential diagnosis of HS include
actinomycosis, anal fistula, cat-scratch disease, cellulitis, inflamed epidermoid cyst,
ischiorectal abscess, lymphogranuloma venereum, nocardiosis, noduloulcerative syphilis,
perirectal abscess, tuberculous abscess, and tularemia [4].

COMPLICATIONS — Long-standing, poorly controlled HS may lead to significant

physical and emotional consequences. Complications include:

●Strictures and contractures [4]

●Lymphatic obstruction, lymphedema of limbs and genitalia

●Malaise, depression, and suicide

●Long-term effects of chronic inflammation including anemia, hypoproteinemia, and

amyloidosis

●Infectious complications (eg, lumbosacral epidural abscess, sacral bacterial osteomyelitis)

[4]

●Arthritis [111]

●Squamous cell carcinoma (SCC)

●Anemia [112]

Fistulae into the urethra, bladder, rectum, and peritoneum are a reported complication of
HS [71]. However, confusion with Crohn disease or coexistence of HS with Crohn disease
may be responsible for these reports.

SCC arising in HS is rare but has been reported in at least 64 cases and appears to be more
common in men [113-119]. A 2011 review of published cases of cutaneous SCC arising in
HS found that SCC primarily occurred 20 to 30 years after the onset of HS (range 2 to 50
years) [118]. Approximately half of these patients died of metastatic disease [118]. Human
papilloma virus infection may be an important contributing factor to the development of
SCC in lesions of HS [117].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines

from selected countries and regions around the world are provided separately. (See "Society
guideline links: Hidradenitis suppurativa".)

SUMMARY AND RECOMMENDATIONS

●Hidradenitis suppurativa (HS) refers to a painful, chronic, suppurative process involving

the skin and subcutaneous tissue. The clinical manifestations of HS appear to result from
infra-infundibular follicular occlusion and secondary rupture of the sebofollicular junction
of folliculopilosebaceous units (FPSUs), resulting in an inflammatory cascade. HS is
neither contagious nor due to poor hygiene. (See 'Pathogenesis' above.)

●HS primarily occurs on intertriginous skin. The axilla is the most common site; the
inguinal area, inner thighs, perineal and perianal areas, mammary and inframammary skin,
and buttocks are additional common sites for involvement. Nonintertriginous skin
involvement also can occur. (See 'Clinical manifestations' above.)

●Initial presentation of HS typically involves recurrent, painful, and inflamed nodules. The
nodules may rupture, discharging purulent, sometimes malodorous material. Persistent
disease leads to coalescence of nodules into inflammatory plaques, epithelialized sinuses,
end-stage "tombstone" comedones, and characteristic "rope-like" scarring. (See 'Clinical
manifestations' above.)

●Diagnosis of HS is based upon the recognition of characteristic clinical manifestations,

including typical lesions in intertriginous sites occurring on a chronic, recurrent basis. A
skin biopsy usually is not necessary for diagnosis. Routine performance of bacterial
cultures is not indicated. (See 'Diagnosis' above.)

●The diagnosis of early stage HS is often missed. The clinician should have a high index of
suspicion for the disease in individuals misdiagnosed with recurrent furuncles or "boils" in
intertriginous areas after puberty. (See 'Diagnosis' above.)

ACKNOWLEDGMENTS

The editorial staff at UpToDate would like to acknowledge Lynette Margesson, MD,
FRCPC, FAAD, who contributed to an earlier version of this topic review.

We are saddened by the death of F. William Danby, MD, FRCPC, FAAD, who passed
away in November 2016. UpToDate wishes to acknowledge Dr. Danby's past work as an
author for this topic.

Use of UpToDate is subject to the Subscription and License Agreement.

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89:709.

Topic 5575 Version 34.0

Surgical management of hidradenitis suppurativa
Author:
Dennis P Orgill, MD, PhD
Section Editors:
Marc G Jeschke, MD, PhD
Mark V Dahl, MD
Deputy Editors:
Kathryn A Collins, MD, PhD, FACS
Abena O Ofori, MD

Contributor Disclosures

All topics are updated as new evidence becomes available and our peer review process is
complete.
Literature review current through: Oct 2018. | This topic last updated: Feb 02, 2017.

INTRODUCTION — Hidradenitis suppurativa (HS) is a chronic, inflammatory skin

disorder of the folliculopilosebaceous units characterized by the development of
inflammatory nodules, pustules, sinus tracts (tunnels), and scars, primarily in intertriginous
areas. Physical pain, odor, chronic drainage, and disfigurement are common features of this
disorder.

The surgical management of hidradenitis suppurativa is reviewed here. The clinical

features, diagnosis, and medical management are discussed separately. (See "Hidradenitis
suppurativa: Pathogenesis, clinical features, and diagnosis" and "Hidradenitis suppurativa:
Treatment".)

GENERAL CONSIDERATIONS — In the setting of refractory HS, surgery may be

needed to remove active foci of disease and eliminate scarred tissue sequelae [1]. Surgery
should not be used in isolation; combining surgery with dietary restrictions and medical
therapy provides the best chance for preventing the development of new lesions and
controlling disease progress. As surgery is an invasive procedure that will result in
additional scarring, prior to proceeding, the risks, benefits, and alternatives should be
discussed with the patient.

Surgical procedures may be performed on individual inflammatory nodules, abscesses, or

sinus tracts and in severe cases may be necessary to excise an entire affected area [2]. The
approach to treating lesions is individualized and becomes more aggressive with higher-
stage HS, particularly if medical therapies have not been successful at controlling the
disease. (See "Hidradenitis suppurativa: Treatment", section on 'Summary and
recommendations'.)

Surgical techniques used to treat HS are listed and discussed in more detail below.

●Punch debridement
●Unroofing

●Wide excision

●Incision and drainage

Surgery aids in the control of inflammation through several mechanisms by removing

epithelialized sinus tracts and associated debris that act as foreign bodies under the skin and
removing and draining inflammatory material. Arresting these processes prevents the
progressive invasion and sinus formation that lead to scarring. (See "Hidradenitis
suppurativa: Pathogenesis, clinical features, and diagnosis".)

PERIOPERATIVE MEDICATION MANAGEMENT

Preoperative treatment — Medical treatment may be administered to calm inflammation

prior to surgical intervention in cases in which the margins of nodules or sinus tracts are
difficult to define. The choice of an anti-inflammatory agent before surgery depends upon
disease severity. (See "Hidradenitis suppurativa: Treatment", section on 'Treatment'.)

●For mild-to-moderate disease (ie, Hurley stage I or II), systemic anti-inflammatory

antibiotics, with or without short courses of prednisone, are used.

●For severe disease (ie, Hurley stage III), systemic glucocorticoids (40 to 60 mg per day
for two to three days followed by a 7- to 10-day taper) or cyclosporine (4 to 5 mg/kg per
day orally) have been used to settle inflammation. Biologic agents, including tumor
necrosis factor-alpha (TNF-alpha) inhibitors, may also be used to calm inflammation in
patients with severe disease prior to surgery.

Antibiotics — The choice and duration of perioperative antibiotics has not been well
studied. For small lesions, many clinicians will continue oral suppressive antibiotics. For
larger lesions, intravenous antibiotics that are culture directed can be useful for several days
following surgery. (See "Hidradenitis suppurativa: Treatment", section on 'Other
antibiotics' and "Hidradenitis suppurativa: Treatment", section on 'Antibiotic therapy'.)

LOCAL PROCEDURES — Local surgical procedures (eg, punch debridement, unroofing)

are used in conjunction with medical therapies for the management of acutely inflamed
nodules and sinus tracts. Based upon observational studies and clinical experience, we
suggest unroofing (local or extensive) initially, rather than wide excision for mild-to-
moderate HS (ie, Hurley stage I or II). For small nodules, we use the technique of punch
debridement (mini-unroofing).

Punch debridement — Punch debridement is our treatment of choice for acute

inflammatory nodules, typically in patients with mild or moderate HS (ie, Hurley stage I or
II). (See "Hidradenitis suppurativa: Treatment", section on 'Hurley stage II'.)
Punch debridement is relatively simple and can be easily performed in the office or clinic
setting. Punch debridement (mini-unroofing) is centered around a single
folliculopilosebaceous unit (FPSU) to evacuate a newly inflamed nodule. The objective of
punch debridement is to remove the fractured FPSU in the initial punch with its associated
sebaceous glands (if still present) and, more importantly, to remove the "bulge" area of the
follicular unit of the FPSU that contains the stem cells hypothesized as responsible for
inducing growth of the proliferative mass and the subcutaneous sinuses and sinus tracts.
The surgical removal of the entire involved FPSU in this manner eliminates the potential
for lesion recurrence and prevents the development of the invasive proliferative gelatinous
mass that results from follicular rupture and likely leads to sinus formation [3]. (See
"Hidradenitis suppurativa: Pathogenesis, clinical features, and diagnosis", section on
'Mechanism'.)

Punch debridement involves the use of a 5- to 7-mm circular punch instrument (identical to
that used in a punch biopsy) to deeply excise the acutely inflamed FPSU within an
inflammatory nodule with a small amount of surrounding tissue. This is followed by
aggressive debridement using digital pressure and then curettage or simple grattage
(scrubbing) with gauze wrapped around a cotton-tipped swab (picture 1). Ferric chloride
can be used for hemostasis, and simple petrolatum dressings are applied after the
procedure. The wound is allowed to heal by secondary intention [4]. Pain relief and healing
are swift.

Unroofing (local or extensive) — Surgical unroofing (also described as deroofing) is

indicated for the treatment of inflamed nodules, abscesses, and sinus tracts typically found
with moderate and severe HS (ie, Hurley stage II or III).

The procedure may be performed on individual nodules or sinuses (local unroofing)

(picture 2 and picture 3) or on all such lesions in an affected region (extensive unroofing)
(picture 4 and picture 5). The technique consists of careful unroofing and debridement of
sinuses and inflamed tissue by a surgeon who has experience with this procedure. Under
local or regional anesthesia, sinus tracts or the inflamed cavity are entered through the
overlying skin or through a sinus opening and opened widely with scissors, explored with
scissor tips or a malleable metal probe, and serially unroofed until no residual tracts of
inflammatory activity remain (movie 1) [5].

The entire roof of each tract should be completely removed and both base and margins
explored for hidden entrances to other sinuses. The active proliferative inflammatory mass
that is attempting to repopulate the area with new FPSUs must be completely removed by
rough gauze grattage (scrubbing) or by sharp curettage with a spoon curette, such as a 6 x 8
mm #0 oval or a Volkmann bone curette. Any residual epithelialized sinus floor may be left
exposed to assist healing by secondary intention, or it may be dissected away [5,6]. Ferric
chloride solution provides adequate hemostasis. Ferric chloride solution for hemostasis (3.8
molal 37.5% weight/volume ferric chloride anhydrous in water, Delasco) is applied with a
cotton swab, then wiped dry, and this is repeated until hemostasis is complete. An
electrocoagulation or electrodesiccation device is very occasionally useful for hemostasis or
for cutting where appropriate.
Carbon dioxide laser excision with healing by secondary intention can also be used to
perform the unroofing of nodules, abscesses, or sinus tracts [7-9]. In one study using this
approach, healing took four to eight weeks and the scars were flat and linear [7]. Only one
recurrence was noted along the margin of the surgical scar in a previously treated area. In a
retrospective review of 185 HS areas in 61 patients, carbon dioxide laser excision appeared
effective for healing areas of HS, with recurrences detected in only two treated sites during
follow-up periods ranging from 1 to 19 years [8].

Role for I&D — Routine incision and drainage (I&D) of individual nodules is not an
effective or appropriate method for managing HS. I&D provides only short-term relief, and
because I&D does not clear the actively growing tissue, lesions treated in this manner tend
to recur [1,2,10]. Thus, we convert any situation that suggests the need for I&D into an
opportunity to perform a punch debridement (for small, acute inflammatory nodules) or an
unroofing procedure (for larger areas of involvement). (See 'Punch debridement' above and
'Unroofing (local or extensive)' above.)

In an early study of 31 patients of whom 6 underwent I&D, the recurrence rate after a mean
follow-up period of 72 months was 100 percent [10]. In a retrospective study of 590
patients with HS who were treated surgically at a single institution between 1976 and 2012,
patients treated with I&D had a higher recurrence risk than those treated with surgical
excision (hazard ratio [HR] 3.5, 95% CI 1.2-10.7) [1]. (See "Technique of incision and
drainage for skin abscess".)

Nevertheless, I&D may be needed for the immediate relief of pain in the setting of a tense
subcutaneous abscess that is too painful to bear. When necessary, the lesion must be deeply
incised. Wide local anesthesia is usually adequate. Packing the wound for a few days is
usually needed to prevent premature superficial closure while the wound fills in from
below. (See "Basic principles of wound management", section on 'Wound packing'.)

The need for repeated I&D should serve as an indicator that initiation of behavioral and
dietary modification and medical therapies to control the disease should be undertaken.
(See "Hidradenitis suppurativa: Treatment", section on 'General approaches'.)

WIDE EXCISION AND RECONSTRUCTION — Wide excision may be needed to

manage an area of chronic or extensive HS (Hurley stage III) when more conservative
medical and surgical measures fail [11]. Surgery, which entails wide excision of the entire
affected area with surgical margins well beyond the clinical borders of disease, combined
with continued aggressive medical management is the treatment most likely to achieve the
best results [2,12]. (See "Hidradenitis suppurativa: Treatment", section on 'Hurley stage III
(severe and refractory disease)'.)

Wide excision can often substantially change the course of disease. Although not
technically curative, many patients will be asymptomatic for long periods of time following
resection [13]. The results are often very satisfying for patients with severe disease that is
not be adequately controlled with medical or less aggressive surgical therapy. Many
patients prefer the more definitive nature of wide excision, particularly if they have been
experiencing complications from medical therapies. There is a tradeoff between performing
a larger excision (with higher surgical complications and longer times to heal) compared
with a smaller excision (with higher risks of developing clinically significant disease
adjacent to the excision). (See 'Recurrence' below.)

It is essential to note that wide excision need not be the destructive "en bloc deep to the
fascia" technique used in managing extensive soft tissue infection or malignancy. Tissue
should be removed until only soft, normal-appearing subcutaneous fat remains. Removing
more than the epidermis, its appendages, sinuses, and associated inflammation and scar
tissue is only necessary when a true fistula (to an underlying hollow organ) is encountered,
which is rare.

The best method of skin closure after wide excision is controversial and largely dependent
on the size of the excision. Some prefer healing by secondary intention for even very large
excisions. Although effective, this submits patients to long periods of dressing changes that
can be painful. Local excision with primary closure (eg, Pollock procedure) is quite useful
for smaller excisions, but if the disease is not adequately excised, active disease can then
present at the periphery of the excision [14]. In one retrospective study of patients who
underwent limited regional excision (resection of abscess and fistula-containing skin in the
affected region [n = 14]) or radical wide excision (removal of all hair-bearing skin in the
affected region with a clear margin of at least 1 cm [n = 11]), the recurrence rates after a
mean follow-up period of 72 months were 43 and 27 percent, respectively [10]. Also, with
local excision and primary closure, wound dehiscence is a risk that increases as the excision
size increases. The trauma of surgery and the tension of the sutures may activate new
lesions along the incision and closure line. We prefer to use mostly subcuticular sutures and
dissolvable sutures on the skin to avoid local reactivation of the disease and to avoid pain of
suture removal.

With more extensive surgical excision, closure of the skin defect with advancement flaps or
split-thickness skin grafting may be needed to accelerate wound healing [15-17]. The
choice of a skin graft versus a flap depends on the size of the lesion; the characteristics of
the adjacent skin; and the age, sex, and body mass index (BMI) of the patient. (See "Skin
grafting and skin substitutes".)

A new concept has been introduced that involves "recycled" skin grafts [18-20]. This has
been applied to large areas of hidradenitis. In these cases, the surgeon harvests the skin
from the resected area, excising the deeper skin and subcutaneous tissue involved in the
inflammatory process and then reapplying the skin as a graft (picture 6).

Axilla — Axillary lesions are one of the most common areas where wide surgical excision
is performed [13]. The extent of the excision varies both in terms of area and depth. These
excisions need to be approached with caution when approaching the area of the axillary
vein since sinus tracts can traverse closely to the vein in the most severe cases.

Primary closure can often be effective for small excisions and for larger excisions in
patients who have lost weight. For wounds with modest tension, the wound can be closed
over a negative pressure wound healing device that is left in place for two to three days.
The patient is then returned to the operating room for removal of the sponge and inspection
of the wound. If clean, the remainder of the wound can be closed [21].

For larger lesions, flaps and skin grafts are often used for closure. Flaps have the advantage
of providing thicker skin coverage; however, the flap can often be too thick and may
require debulking.

When skin grafts are used, they are often meshed to help with the contour of the wound and
allow for drainage. Skin grafting is also quite effective but takes longer to heal compared
with flaps. For very large areas, flaps are very often not an option, and skin grafts remain a
nice option.

Inguinal/perineal — Lesions commonly also occur in the groin (picture 7) and can also
occur in the perineum, labia majora, and scrotum. Sinus tracts in this area can be extensive,
and meticulous dissection is required to get a clear removal. In the scrotum, these can track
around the spermatic cord. Judgment is needed to determine the extent of excision and type
of closure. We follow similar guidelines for closure in the axilla (above).

Gluteal — Gluteal lesions can often be extensive and can either involve the skin or in some
cases have extensive subcutaneous tunneling. Radical excision of the tunnels and diseased
tissue is necessary to clear the disease.

Perianal — Perianal lesions are challenging as they are near the anus. Local flaps and skin
grafts are often necessary to provide coverage in these areas. We generally defer surgical
treatment in this region until other areas of the body have been treated. In some cases,
lesions located here improve as systemic inflammation from other areas subsides.

Breasts and lower abdomen — Disease on the underside of large breasts and beneath a
pannus is frequently seen in obese patients. These lesions can be treated with good
outcomes using breast reduction surgery or panniculectomy. (See "Overview of breast
reduction".)

POSTOPERATIVE CARE AND FOLLOW-UP

Dressings — Postoperatively, dressings are applied using the principles discussed in the
preceding sections.

Generally, for unroofed wounds, simple plain petrolatum is applied to gauze in a very thick
(4- to 5-mm) layer, and the resulting nonadherent dressing, laid on or bound to the wound,
will suffice as a postsurgical dressing. The petrolatum must cover all surfaces of the gauze
to avoid adherence of dry gauze to the wound. Daily (or more frequent) dressing changes
involve gentle rinsing of the wound in clear running water, free of soap or cleansers,
followed by the reapplication of petrolatum. The gauze is best eliminated from the wound
care regimen as soon as possible to avoid scrubbing off newly generated epithelium. The
epithelializing margins of the wound must not be debrided or even touched. Loose clothing
should be worn to avoid friction in the reepithelializing areas.
After the initial few days, a simple thick application of petrolatum to the wound edges is
sufficient. Healing progresses inward from the wound edges and upward from the wound
base (picture 8) and may be accelerated in large wounds with skin grafting [22,23].

Negative pressure wound therapy — Negative pressure wound therapy is our preferred
method for managing large open wounds and facilitating adherence of skin grafts or flaps.
It has been used successfully in the management of wounds resulting from severe HS [24].
(See "Negative pressure wound therapy".)

COMPLICATIONS — Complications from surgery can include bleeding, infection-

delayed healing, and new disease at the periphery. Resection wounds are highly
contaminated, and, if wound complications occur, a combination of intravenous antibiotics
and negative pressure wound therapy can be used to manage them.

Recurrence — In a systematic review, the estimated average recurrences were 22 percent

with local incision, 27 percent with unroofing, and 13 percent with wide excision (15
percent for primary closure, 8 percent for flap closure, and 6 percent for graft closure) [4].
Although many authors use the term "recurrence," in our experience, the disease does not
"recur" after wide excision but can develop in other areas, either around the previous
excision or in other anatomic areas. If disease comes back adjacent to areas that are widely
excised, we will generally refer the patient for medical management and try to space
surgeries at least three months apart. If medical management is not successful, in most
cases small excisions with direct closure can be used to treat any new disease.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines

from selected countries and regions around the world are provided separately. (See "Society
guideline links: Hidradenitis suppurativa".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education

materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are
written in plain language, at the 5th to 6th grade reading level, and they answer the four or
five key questions a patient might have about a given condition. These articles are best for
patients who want a general overview and who prefer short, easy-to-read materials. Beyond
the Basics patient education pieces are longer, more sophisticated, and more detailed. These
articles are written at the 10th to 12th grade reading level and are best for patients who want
in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to
print or e-mail these topics to your patients. (You can also locate patient education articles
on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Hidradenitis suppurativa (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Successful treatment of hidradenitis suppurativa (HS) requires lifelong attention to
prevent new lesions in all stages of the disease. Measures to prevent new HS lesions
include dietary and behavioral changes and topical or systemic medications, including
antibiotics. Surgical interventions may become necessary to manage nodules, abscesses,
sinus tracts, and scars. (See 'Introduction' above.)

●Surgery should not be used in isolation; combining surgery with dietary restrictions and
medical therapy provides the best chance for preventing the development of new lesions
and controlling disease progress. (See 'General considerations' above and "Hidradenitis
suppurativa: Treatment".)

●Surgical options for management of HS include punch debridement, unroofing of sinus

tracts or abscesses, and wide excision. The choice of procedure depends on the severity of
the lesion. The evidence supporting these interventions is limited to observational studies
and clinical experience. Our approach is generally as follows (see 'General considerations'
above):

•For acutely inflamed nodules or extensive sinuses, we suggest unroofing (local or

extensive) combined with medical therapy, rather than wide excision (Grade 2C). For small
nodules, we use the technique of punch debridement (ie, mini-unroofing). (See 'Unroofing
(local or extensive)' above.)

•For patients with chronic or extensive disease, we suggest wide excision of the entire
affected area combined with continued aggressive medical management (Grade 2C). Wide
excision offers the best chance to provide permanent relief for patients with HS in a
localized area that fails to respond sufficiently to medical therapies and that has progressed
to a point where surgical unroofing has failed or is unlikely to be sufficient. Some patients
with earlier stages of disease may request surgical excision if they become frustrated or
have complications related to medical therapies. (See 'Wide excision and reconstruction'
above.)

•We suggest not performing incision and drainage (I&D) alone for the management of HS
except in the setting of a tense abscess when immediate relief of pain is required (Grade
2C). I&D alone is not an effective method for managing HS because lesions treated in this
manner tend to recur. (See 'Role for I&D' above.)

ACKNOWLEDGMENTS

The editorial staff at UpToDate would like to acknowledge Lynette Margesson, MD,
FRCPC, FAAD, who contributed to an earlier version of this topic review.

We are saddened by the death of F. William Danby, MD, FRCPC, FAAD, who passed
away in November 2016. UpToDate wishes to acknowledge Dr. Danby's past work as an
author for this topic.

Use of UpToDate is subject to the Subscription and License Agreement.

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