Hidradenitis Supurativa

Christos C. Zouboulis & Fragkiski Tsatsou

Fitzpatrick’s Dermatology and General Medicine
8th Ed 2012
Page 953 - 9

Presented By : Dr. Elfa Wirdani Fitri, M.Kes, Sp.KK

 HS = Acne Inversa
• HS is a chronic, inflammatory, recurrent,
debilitating skin disease (of the hair follicle)
• usually presents after puberty with painful,
deepseated, inflamed lesions in the apocrine
gland-bearing areas of the body
• most commonly the axillary, inguinal, and
anogenital regions
 Etiology & Pathogenesis
• HS is a disease of the terminal hair follicle
associated with lymphohistiocytic
inflammation, granulomatous reactions, sinus
tracts, and scarring
• A consistent finding in histological studies of
HS is a follicular occlusion due to
hyperkeratosis, regardless of disease duration,
whereas an isotopic hyperplasia of follicular
epithelium is evident
 Etiology & Pathogenesis
• This leads to occlusion of the apocrine gland
with subsequent follicular rupture,
perifollicular inflammation and possible
secondary infection, giving rise to clinical
findings
1. ADNEXAL STRUCTURES
• HS was thought to represent a primary
disorder of apocrine glands, and was also
referred to as apocrinitis
• Shelley and Cahn provided additional support
of this concept by hypothesizing that poral
occlusion of the apocrine duct reproduced the
clinical and pathologic lesions using their
experimental model
• More recent publications have refuted the
concept that this primary event in HS is
apocrine gland inflammation and postulate
that apocrine glands become secondarily
affected
2. GENETIC FACTORS
• A family history of HS may be elicited in 26%
of patients
• Several studies have not demonstrated HLA
associations
• Others studies have suggested an autosomal
dominant mode of inheritance with single
gene transmission
3. ASSOCIATED DISEASES
• A pilot study carried on ten HS patients
detected no CARD15/NOD2 polymorphisms,
found to be associated with Crohn disease
• Other reported associations include pyoderma
gangrenosum, nephrotic syndrome, and
amyloidosis, Dowling–Degos disease, and
arthropathy
• A current systematic literature review
reported that follicular occlusion disorders,
inflammatory bowel diseases, especially Crohn
disease, spondyloarthropathy, other
hyperergic diseases, genetic keratin disorders
associated with follicular occlusion and
squamous cell carcinoma are the most
common HS comorbid diseases
4. HORMONES AND ANDROGENS
• The tendency of HS to develop at puberty or
postpuberty suggests an androgen influence
• Additionally, disease flares have been
reported postpartum, in association with the
oral contraception pill, and in the
premenstrual period (approximately 50% of
patients)
• Antiandrogen therapy has also demonstrated
therapeutic benefit in some studies
• However, no biochemical evidence of
hyperandrogenism was found in 66 women
with HS.
• Additionally, unlike the sebaceous glands, the
apocrine glands are not affected by androgens
• Thus, the influence of androgens on HS is
unclear.
5. OBESITY
• Obesity is unlikely to be a causal factor in HS
but is often regarded as an exacerbating factor
by increasing shearing forces, occlusion,
keratinocyte hydration, and maceration
• Obesity may also exacerbate disease by
creating a state of androgen excess
• Weight reduction is recommended for
overweight patients and may help to control
disease
6. BACTERIAL INFECTION
• Bacterial involvement is thought by some
authors to occur secondarily
• Routine cultures are often negative, but
numerous bacteria have been recovered from
lesions
7. SMOKING
• One study determined that 70% of their 43
patient cohort with perineal HS were smokers
• It is postulated that smoking affects
polymorphonuclear cell chemotaxis
• Smoking cessation may improve the clinical
course of the disease
 ETIOLOGY

Bacteria Obesity

Genetic Stress
HIDRADENITIS
SUPPURATIVE
PATHOGENESIS Other
Genetically carries autosomal possible
dominant gene etiology
Exacerbating Associated
Follicular hyperkeratosis disease
factors
(obesity, Hormon &
smoking, Follicular occlusion androgens
stress) influence
Follicular rupture Bacterial
infection
Inflammation

Formation of sinus tract

Secondary infection

Healing: Scarring & contractures

 CLINICAL FINDINGS
• One of the most obvious hallmarks of the disease
is the restriction to the skin areas affected
• The disease is essentially limited to the
intertriginous areas, although aberrant lesions
may occur
• The sites affected in order of decreasing
frequency include: axillary, inguinal, perineal and
perianal, mammary and inframammary, buttocks,
pubic region, chest, scalp, retroauricular, and
eyelid
• HS is not acne: closed comedones are not
seen, since the deep part of the follicle
appears to be involved and not its superficial
compartments, as seen with acne affecting
convex skin surfaces
• HS inflammatory lesions are initially transient,
but gradually become intransigent and
associated with significant scarring
 SEVERITY
• Three stages of disease are recognized and
named after Hurley (I–III)
 In the primary stage, abscesses develop in
isolated locations
 The secondary stage involves the development of
sinus tracts with scars bridging individual lesions
 The tertiary stage shows coalescing lesions with
scarring and sinus tracts, inflammation, and
chronic discharge
• The disease onset is insidious and shows
variable severity.
• Otherwise healthy postpubertal individuals
initially may experience slight discomfort or
pruritus.
• After this, a tender papule or deep-seated
nodule (0.5 to 2.0 cm) ensues.
• Pustules may also be visualized.
• This nodule may slowly resolve or may expand
and coalesce with surrounding nodules to
form large painful inflammatory abscesses.
• These abscesses are rounded without central necrosis and
may resolve or rupture spontaneously, producing a purulent
discharge (Fig. 85-3)
• Eventual healing may result
in scarring with fibrosis (Fig.
85-4), dermal contractures
and rope-like elevation of the
skin (Fig. 85-5), and double-
ended comedones
• Sinus tracts may also develop (Fig. 85-6)
DIFFERENTIAL DIAGNOSIS

FURUNCULOSIS

SEBACEOUS CYST

SKROFULODERMA
 LABORATORY TESTS
• Patients with acute lesions of HS may
demonstrate an increase in the erythrocyte
sedimentation rate or C-reactive protein
• If there is any concern over infection, then
deep cultures (not skin surface) from lesions
should be conducted and submitted for
bacterial, tuberculosis, and fungal cultures
 SPECIAL TESTS
• Histopathology
• USG
• MRI
Microscopic photo showing an epithelial cyst
with focal acute inflammation (black arrow).
 COMPLICATIONS
• QUALITY OF LIFE (Relate to disease-associated
pain)
• SYSTEMIC COMPLICATIONS
(Septicemia,Anemia,Leukocytosis)
• LOCAL COMPLICATIONS (Scarring may limit
mobility. Anal, urethral, or rectal strictures, Urethral
fistulas. Persistent penile, scrotal, or vulvar
lymphedema due to blockade or destruction of local
lymph drainage routes. Squamous cell carcinoma
(SCC))
 TREATMENT
• The objective of patient management is
prevention of the development of primary lesions
as well as resolution, amelioration, or regression
of secondary disease features such as scarring or
sinus tract formation
• Hurley :
stage I is manageable with systemic drugs,
stage II may benefit from medical treatment and
from limited excisions of locally recurring lesions,
stage III requires radical surgery
• Thirty-two experienced complete remission of
HS of between 1 and 4 years after only one
course of treatment, and further two patients
achieved remission after substituting
clindamycin with minocycline (100 mg/day)
because of transient diarrhea.
• Twenty-one patients were unable to complete
the course of treatment because of side
effects, mostly diarrhea.
• Intralesional corticosteroids may be of benefit for
patients with an isolated number of tender
lesions.
• Oral isotretinoin is ineffective in the treatment of
HS.
• Case reports or small case series have also shown
therapeutic success with other systemic
therapies, including systemic corticosteroids,
azathioprine, cyclosporine, dapsone, and
methotrexate
• A small case series demonstrated the efficacy
of treatment of persistent painful nodules
with cryotherapy.
 Other Tx
• SURGERY
• RADIOTHERAPY
 PREVENTION PHARMACOLOGY

• Stop smoking • Topical antibiotic

• Loss body weight • Systemic antibiotic
• Wear lose clothes • Systemic
corticosteroid
• Intra-lesional
corticosteroid
• Hormonal therapy
• Retinoid
• Immunosuppressor
• Biologic agent
 PSYCHOLOGICAL
LASER THERAPY SURGERY THERAPY
THERAPY
• Radiotherapy • Wide excision and • hidradenitis
• Cryotherapy skin-grafting information
• CO2 laser • Skin flap transfer development and
• Closure primer exchange (HIDE)
• Nd:YAG laser
• Fasciocutaneous or
musculocutaneous
flap