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Indian J Dermatol
v.54(1); Jan-Mar 2009
PMC2800861
Abstract
Introduction
Erythroderma is an intense generalized redness of the skin; it was first described by Von
Hebra in 1868. It is an inflammatory disorder characterized by an extreme state of skin
dysmetabolism that gives rise to extensive erythema and scaling all over the body. This
condition classically involves greater than 90% of the body surface. The erythrodermic state
is of great concern because it poses significant risk of morbidity and mortality, in addition to
the risks inherent to the underlying disease and its therapy.
Erythroderma can be fatal, even when properly managed, primarily because of its metabolic
burden and complications. Hence it is mandatory to establish its etiopathology in order to
facilitate precise management. This disorder may be the morphologic presentation of a
variety of cutaneous and systemic diseases, and a thorough workup is essential. A detailed
outline of the patient's history to elicit possible triggering events, including infections, drug
ingestion, topical application of medications, sun/ultraviolet exposure and other factors,
should be determined. Management of the skin disorder continues to be a challenge due to
its multiple etiologies. The prognosis of erythroderma is determined by its underlying cause.
There is a paucity of information on erythroderma in Africa. The growing increase in use of
herbal medicines,[1] the HIV epidemic,[2] hospital visits in advanced disease stages and
limited investigational resources in Third World environments all exacerbate the lifethreatening nature of this condition. With increasing use of herbal and nonherbal medicines,
more individuals are at risk of contracting erythroderma.
Go to:
Figure 1
Fifteen-years-old girl with diffuse erythema and scaling of lower extremities
Figure 2
Sloughing of epidermis of the soles in the above patient
Case 2
A 64-year-old man presented to our Benin City clinic in 2005 with an acute eruption of
numerous erythematous plaques that had appeared 2 weeks following oral intake of an
unknown amount of Aloe vera leaves taken to enhance well-being. There was no history of
preexisting dermatologic or medical conditions. Physical examination revealed scaling
plaques with prominence on the scalp, trunk and extremities [Figure 3, Figure 4]. Laboratory
investigations were consistent with dehydration and inflammatory changes. Topical and oral
steroids achieved rapid improvement of the lesions.
Figure 3
Sixty-four-years-old man with papulosquamous plaques on the scalp, arms and trunk
Figure 4
Erythematous scaling plaques of the lower extremities
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Epidemiology
The true incidence of erythroderma is unknown. Gehgal and Srivastava[3] performed a large
prospective study in the Indian subcontinent, where they determined the incidence to be 35
per 100,000 dermatologic outpatients. Sigurdsson[4] recorded the annual incidence in the
Netherlands to be 0.9 per 100,000 inhabitants. In general, studies have shown a male
predominance, with the male-to-female ratio ranging from 2:1 to 4:1, and the mean age
between 40 and 60 years.[3] Rym et al. conducted a retrospective study of 80 erythrodermic
adults, looking at patients examined between 1981 and 2000.[5] Patient information included
clinical, laboratory, histopathological and therapeutic data. The incidence of erythroderma
from the study was 0.3%, the average age being 53.78 18 years; and the male-to-female
ratio, 2.2:1. These numbers may, however, underestimate the current statistics in Third
World environments such as those in Africa.
Go to:
Etiology
A major challenge lies in establishing the underlying cause of erythroderma. Most published
series reveal that the majority of patients are diagnosed with psoriasis, spongiotic dermatitis,
drug reactions or cutaneous T cell lymphoma (CTCL).[6,7]
A preexisting dermatosis is the single most common cause of adult erythroderma.[3,5,813]
A number of dermatoses can progress to erythroderma, but the most common include
psoriasis and eczema.[3,5,9,12] Rym et al. reported 41 of 80 erythrodermic patients had
psoriasis,[5] a finding not inconsistent with Spanish, Middle Eastern and Indian
studies.[3,9,12] Psoriatic erythroderma may occur in relation to withdrawal of systemic or
topical glucocorticoids, use of systemic medications such as lithium and antimalarials,
phototherapy burns, infection and systemic illnesses.[14]
The apparent increase in the incidence of exfoliative dermatitis may have a bearing on the
introduction of many new drugs. It is therefore important to consider all drug
exposures.[14,15] Patients presenting with morbilliform, lichenoid or urticarial drug eruptions
may develop generalized exfoliative dermatitis.[16] This association has been inferred from
descriptions of patients on antiepileptic medications, antihypertensive agents, antibiotics,
calcium channel blockers and a variety of topical preparations.[14,17] Severe exfoliative
erythrodermic dermatitis has been reported with use of proton pump inhibitors.[18] Morar[19]
identified adverse drug reactions to antituberculosis medication as the most common cause
of erythroderma in HIV-seropositive South African patients. Rym et al.[5] implicated
carbamazepine, phenobarbital, penicillin and paracetamol. The intake of herbal medicines,
being very popular, may also increase risk among Africans.
Erythroderma may be a cutaneous manifestation of malignancy. The incidence of internal
malignancy is approximately 1%.[20] Reticuloendothelial neoplasms, as well as internal
visceral/blood vessel malignancies, may manifest as erythroderma.[16,21] Laryngeal, thyroid,
lung, esophageal, gallbladder, gastric, colon, fallopian tube and prostate carcinomas and
lymphomas have all been implicated.[2229] Other associations include cutaneous T cell
lymphomas, which comprise mycosis fungoides and Szary syndrome.[20,3032] Mycosis
fungoides may progress from, accompany, or follow T cell lymphomas, and their presentation
may be similar to benign erythroderma.[16,30,33,34] The ability to distinguish malignant from
benign erythroderma may require an immunophenotypic study with the use of advanced
antibody panels.[35] Definitive diagnosis of erythroderma due to internal malignancy cannot
be made based on clinical presentation alone, but a concomitant history of insidious
development, progressive decompensation, refractoriness to standard therapeutics and
absence of prior skin pathology may be existent.[28]
Erythroderma is also associated with disorders that are not readily classified. It has been
reported in association with dermatophytosis, hepatitis, renal failure, acquired
immunodeficiency syndrome, congenital immunodeficiency syndrome (Omenn syndrome),
graft-versus-host disease, histoplasmosis, lupus, dermatomyositis, thyrotoxicosis and
sarcoidosis.[3647]
Go to:
Pathogenesis
Currently, the mechanism of erythroderma is unclear. Adhesion molecules and their ligands
play a significant role in endothelial-leukocyte interactions, which impact the binding,
transmigration and infiltration of lymphocytes and mononuclear cells during inflammation,
injury or immunological stimulation.[48] The rise in adhesion molecule expression (VCAM-1,
ICAM-1, E-selectin and P-selectin) seen in exfoliative dermatitis stimulates dermal
inflammation, which may lead to epidermal proliferation and increased production of
inflammatory mediators.[48] The complex interaction of cytokines and cellular adhesion
molecules such as interleukin-1, -2 and -8; intercellular adhesion molecule-I (ICAM-I); and
tumor necrosis factor (TNF)[49] results in significantly elevated epidermal turnover rate,
leading to above-normal mitotic rate.[50] The amount of germinative cells increases and the
transit time of keratinocytes through the epidermis decreases, causing loss of more cellular
material from the surface.[14]
Sigurdsson et al. conducted an immunohistochemical study and observed that the dermal
infiltrate in patients with Szary syndrome mainly showed a T-helper 2 (Th2) cytokine profile,
in contrast to a T-helper 1 (Th1) cytokine profile in benign reactive erythroderma, which
suggests that a relatively uniform clinical picture in erythroderma does not imply similar
pathomechanisms for various etiologies.[48,51]
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Clinical Features
The pattern observed is erythematous patches, which increase in size and coalesce to form
extensive areas of erythema, and eventually spread to involve most of the skin
surface.[50,52] Some studies have shown sparing of the nose and paranasal areas, and this
has been described as the nose sign.[53,54]
The epidermis appears thin, giving a glossy appearance to the skin. Once erythema has
been established, white or yellow scales develop that progress to give the skin a dry
appearance with a dull scarlet and gray hue.[14] Induration and thickening of the skin from
edema and lichenification may provoke a sensation of severe skin tightness in the
patient.[14] The skin is bright red, dry, scaly and warm to touch.
Some patients may experience involvement of their palms and soles, with hair loss and nail
shedding.[55] Involved nails are thick, lusterless, dry, brittle, and show ridging of the nail
plate.[50] Subungual hyperkeratosis, distal onycholysis, splinter hemorrhages occur; and
sometimes, the nails may shed.[14] Shelley[56] described alternating bands of nail plate
discontinuity and leukonychia in drug-induced erythroderma.
Sometimes, the clinical presentation may be suggestive of the underlying cause. Typical
psoriasiform plaques may be apparent in early erythrodermic psoriasis. Pityriasis rubra pilaris
shows islands of sparing, orange-colored palmoplantar keratoderma and hyperkeratotic
follicular papules on juxta-articular extensor surfaces.[14] The violaceous papules and
reticulated buccal mucosal lesions of lichen planus may be evident. Joly et al.[57] described
three African patients presenting with lichenoid erythroderma different from the classic form
of lichen planus pemphigoides. The presence of heavy crusts on the palms and soles with
subungual hyperkeratosis raises the possibility of Norwegian scabies.[14] In patients with
pemphigus foliaceus, crusted patches and erosions may appear on the face and upper trunk.
A heliotrope rash, poikiloderma, Gottron's papules, periungual telangiectases and muscle
weakness may be seen in erythrodermic dermatomyositis.[26,42] Papuloerythroderma of
Ofuji presents in elderly men as flat-topped red papules that become generalized
erythrodermic plaques without the involvement of abdominal skin folds (deck chair
sign).[5860]
Laboratory Findings
Laboratory findings in the erythrodermic patient are usually nonspecific.[16] Common
abnormalities are mild anemia, leukocytosis with eosinophillia, elevated sedimentation rate,
decreased serum albumin, increased uric acid, abnormal serum protein electrophoresis with
polyelevation in the gamma globulin region and elevated IgE levels.[49,61] Eosinophilia is
generally a nonspecific finding, although a highly elevated count raises the possibility of a
lymphoma.[52] Circulating Szary cells may be present; but whereas less than 10% is
considered nonspecific in the setting of erythroderma, the presence of 20% or more raises
the suspicion for Szary syndrome.[13,62]
Go to:
Histopathology
Biopsy specimens tend to have many nonspecific features.[16] Hyperkeratosis,
parakeratosis, acanthosis and a chronic perivascular inflammatory infiltrate, with or without
eosinophils, are examples.[6] The clinicopathologic correlation is difficult because nonspecific
features of erythroderma may mask the specific features of an underlying dermatosis. Direct
immunofluorescence studies may be of diagnostic utility in cases of erythroderma secondary
to pemphigus foliaceus, bullous pemphigoid, graft-versus-host disease and connective tissue
disorders.[52]
Go to:
Management
The initial management of erythroderma is the same regardless of etiology. This should
include replacement of nutritional, fluid and electrolyte losses.[6] Local skin-care measures
should be employed, such as oatmeal baths as well as wet dressings to weeping or crusted
sites followed by the application of bland emollients and low-potency corticosteroids.[63]
Known precipitants and irritants are to be avoided; and underlying cause, with its
complications, is to be treated.[6,16,50] Secondary infections are treated with antibiotics.
Edema in dependent areas, such as in periorbital and pedal areas, may require diuretics.[63]
Hemodynamic or metabolic instability should be addressed adequately. Serum protein,
electrolyte and blood urea levels should be monitored. This condition may resist therapy until
the underlying cause is treated; hence it is important to determine underlying etiology early in
its management.[63,64] In Africa and other Third World environments, however, this may not
be possible.
Go to:
Course
The disease course is greatly influenced by etiology. It is progressive when due to drug
allergy, lymphoma, leukemia, contact allergens or staphylococcal scalded skin
syndrome.[3,6,13,50] A slower course is observed if from a primary skin disease such as
psoriasis or atopic dermatitis.[3,6,13,50] Drug-induced erythroderma patients recover
completely with prompt diagnosis and treatment.[6,50] The outcome is unpredictable in
idiopathic erythroderma, and its course is marked by multiple exacerbations; prolonged
corticosteroid use is often required.[16,50,62]
Go to:
Prognosis
Conflicting reports have been published regarding the prognosis of patients with
erythroderma in developed nations. The most common causes of death in patients with
erythroderma are pneumonia, septicemia and heart failure.[16,63] Elderly patients who
develop complications such as infection, fluid/electrolyte imbalances and cardiac failure are
at higher risk of mortality.[16,50] Initial studies record death rate in the range of 4.6% to
64%,[5,9,16,49,50,61] but this has since been reduced due to advancement in diagnosis and
therapy.[50] In Third World countries, such as parts of Africa, the former prognosis may be
more accurate.
Go to:
Footnotes
Source of Support: Nil
Conflict of Interest: Nil.
Go to:
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Erythroderma. A clinical and follow-up study of 102 patients, with special emphasis
on survival.[J Am Acad Dermatol. 1996]
Sigurdsson V, Toonstra J, Hezemans-Boer M, van Vloten WA
J Am Acad Dermatol. 1996 Jul; 35(1):53-7.
Erythroderma. A clinical and follow-up study of 102 patients, with special emphasis
on survival.[J Am Acad Dermatol. 1996]
Sigurdsson V, Toonstra J, Hezemans-Boer M, van Vloten WA
J Am Acad Dermatol. 1996 Jul; 35(1):53-7.
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