EXFOLIATIVE DERMATITIS AT A GLANCE

Exfoliative dermatitis ( ED ) is defined as diffuse erythema and scaling of the

skin involving more than 90 % of the total body skin surface area.
Systemic and potentially life-threatening complications . include fluid and
electrolyte imbalance, thermoregulatory disturbance, fever, tachycardia,
high-output failure, hypoalbuminemia, and septicemia.
Common underlying etiologies are psoriasis, atopic, dermatitis, and other
spongiotic dermatoses, drug hypersensitivity reaction, and cutaneous T-cell
lymphoma ( CTCL ). The cause of ED is unknown ( idiopathic ) in
approximately 20 % of cases.
Diagnostic workup includes a complete history and physical examination,
with careful analysis of pertinent clinical clues and dermatohistopathology.
Other laboratory workup is often required and determined by clinical clues.
Management of ED involves combining symptomatic relief with addressing
the underlying etiology and potential
Prognosis is variable and depends primarily on the underlying etiology. Druginduced ED has the best prognosis while malignancy-associated ED has the
highest mortality.

EPIDEMIOLOGY
Several large studies have reported a widely incidence of exfoliative dermatitis
( ED ) ranging from 0.9 to
71.0 per 100,000 outpatient. A male predominance has been described, with a
male-to-female ratio of approximately 2 : 1 to 4 : 1. Any age group can ben
affected, and with most studies excluding children, the average age of disease
onset varies from 41 to 61. ED is a rare disease in children, and only little
epidemiologic data is available for pediactric populations. One study found 17
patients, recorded over a 6 year period, with a mean age of onset of 3.3 years and a
male-to-female ratio of 0.89 : 1. ED occurs in all races.
A preexisting dermatosis plays a role in more than one-half of the cases of ED.
Psoriasis is the most common underlying skin disease ( almost one-fourth of the
cases ). In a recent study of severe psoriasis, ED was reported in 87 of 160 cases
ETIOLOGY AND PATHOGENESIS
Eatabilishing the etiology of ED can be challenging since it can be caused by a
variety of cutaneous and systemic diseases. A compilation 0f 18 published studies
from various countries on ED shows that a preexisting dermatosis is the most
frequent cause in adults ( 52 % of ED cases ; range, 27 % - 68 % ) followed by drug
hypersensitivity reactions ( 15 % ), and cutaneous T-cell lymphoma ( CTCL ) or
sezary syndrome ( 5 % ). No underlying etiology is identified it approximately 20 %
of ED cases ( range, 7 % - 33 % ) and these cases are classified as idiopathic.
Psoriasis is the most common underlying dkin disease to cause ED ( 23 % of
cases ), followed by spongiotic dermatitis ( 20 % ). Possible triggers for psoriatic ED
include the following :

Medications, such as lithium, terbinafine, and antimalarials

Topical irritants including tars
Systemic illiness
Discontinuation of potent topical or oral corticosteroids, methotrexate, or
biologics ( efalizumab )
Infection including human immunodeficiency virus ( HIV )
Pregnancy
Emotional stress
Phototherapy burns
Less commen causes of ED in adults include immunobullous disease; connective
tissue disease; infections, including scabies and dermatophytes; pityriasis rubra
pilaris ( PRP ) ( 4 % of dermatoses ); and underlying malignancy. Even in patients
with underlying dermatoses, it is critical to consider other possible etiologies. In one
case series, malignancy-related ED was identified in seven patients, five of whom
had a preexisting dermatosis. In about 5 % - 10 % of idiopathic ED cases,
erythrodermic CTCL was ultimately diagnosed. Solid organ malignancies as well as
hematologic and reticuloendothelial malignancies may manifest as ED.
In neonates and infants, the differential diagnosis includes dermatoses ( such as
psoriasis, atopic dermatitis, and seborrheic dermatitis ), drugs, and infection
( particularly staphylococcal scalded-skin syndrome ). In additions, several
congenital disolders including the ichthyoses, both bullous and nonbullous
congenital ichtyosiform erythoderma, netherton syndrome, and immunodeficiencies
should be considered ( box 23-1 )
BOX 23-1 DIFFERENTIAL DIAGNOSIS
Most likely
Spongiotic dermatitis ( 20 % - 24 % ) ( atopic, 9 % ; contact dermatitis, 6 %;
seborrheic dermatitis, 4 % chronic actinic dermatitis, 3 % )
Psoriasis ( 23 % )
Drug hypersensitivity reaction ( 15 % )
Cutaneous T-cell lymphoma ( 5 % )
Idiopathic ( approximately 20 % )
Consider
Contact dermatitis
Immunobullous disease ( superficial pepmhigus, bullous pemphigoid,
paraneoplastic pemphigus )
Infection ( scabies, dermathophytosis )
Toxin-mediated ( toxic shock syndrome, staphylococcal scalded-skin
syndrome )
Chronic actinic dermatitis
Pityriasis rubra pilaris
Collagen vascular disease
Paraneoplastic ( solid tumors and hematologic )
Primary immunodeficiency
Congenital ichthyoses
Always rule out

Cutaneous T-cell lymphoma

Drug-induced hypersensitivity syndrome
Paraneoplastic

Topical and systemic medications are implicated in a significant percentage og

ED cases ( 15 %; range,4 % - 39 % ) and the introduction if new drugs is likely to
increase the incidence of ED. Both allopathic and naturopathic medication have
been suggested to cause ED and the list is constantly expanding. The most
commonly implicated drugs include calcium channel blockers, antiepileptics,
antibiotics ( penicillin family, sulfonamides, vancomycin ), allopurinol, gold, lithium,
quinidine, cimetidine, and dapsone. However, most of the drugs are reported in
single case reports. In addition to drugs, the contrast medium iodixanol
( visipaque ) used during percutaneous coronary intervention has recently been
reported to cause ED.
Currently, the pathogenic mechanism of ED have not been elucidated. It is not
well undestoot how a preexisting dermatosis progresses to ED, an underlying
disease manifest as ED, or the de novo ED develops. While the clinical presentation
is similar in patiens with diverse etiologies of ED, it is likely that different pathways
lead to the common end result of skin-selective recruitment of inflammatory cells.
Cytokines, chemokines, and their receptors are believed to play an important
role in the pathogeness of ED. A study of cytokine profile in dermal infiltrates
showed that there may be differences in pathopysiologic mechanisms between
benign ED and sezary syndrome-a T helper 1 cytokine profile was found in benign
ED while a T helper 2 cytokine profile was found in sezary syndrome. In a recent
report, an overexpression of both T helper 2-related chemokine receptors ( CCR4,
CCR5
CUTANEOUS LESIONS
The calassic presentation of ED is erythematous patches that increase in size and
coalesce into generalized red erythema with a shiny appearance. By definitions, ED
involves more than 90 % of the patient skin surface. A few days after onset of
erythema, fine white or yellow calling begins, classically arising in the flexures.
Plate-like scaling may occur acutely and on the palms and soles. The scalling
progresses further, giving the skin a dull red appearance. With chronicity, edema
and lichenification lead to skin induration. Ectropion and epiphora may develop
secondary to chronic periorbital involvement. Palmoplantar keratoderma has been
noted in up to 80 % of patient with chronic ED.
LABORATORY TEST
Laboratory test are most often not diagnostic and not specific. Common
laboratory abnormalities found in ED patient include anemia, leukocytosis,
lymphocytosis ,eosonophilia, increased IgE , descreaced serum albumim, and an
elevated erythrocyte sedimentation rate. Fluid loss may lead to electrolyte
abnormalities and abnormal renal function ( elevated creatinine level ). Elevated
IgE levels have been noted in patient s with ED unrelated to atopic dermatitis

including in 81.3 % of psoriatic ED patients. Eosinophilia is nondiagnostic and has

been found in 20 % of ED patients. However, when highly elevated eosinophil
counts are noted, the possibility of associated Hodgkin disease must be
investigated.
It is very important to differentiate benign erytrodermic inflammatory diseases
from sezary syndrome. In cases where erythodermic CTCL is suspected, a throught
evaluation of skin, blood, and lymph node samples is required for definitive
diagnosis. Studies have shown that a level of 20 % or more circulating sezary cells
is a useful diagnostic criterion for sezary syndrome, whereas less than 10 % is non
specific. Exeptions do occur, such as in certain severe drug induced reactions that
can mimic sezary syndrome ( as hydantoin hypersensitivity ). Several benign
dermatoses, including psoriaris, atopic dermatitis discoid lupus, lichen planus, and
parapsoriaris show the precence of sezary cells in numbers less 10 %.
Demonstration of a clonal T cell receptor gene rearrangement is recommended for
a sensitive and specific differentiation of sezary syndrome from other etiologies ED.
HISTOLOGIC CLUES OF UNDERLYING DISEASE

TREATMENT
Topical
Emollients ( water in oil emulsion )
Keratolystics ( salicylic acid, urea )
Vitamin D3
Physical
Photochemotherapy ( topical or systemic PUVA )

 Extracorporeal photopheresis
Systemic
Retinoids ( 0.5 0,75 mg/kg acitretin/day )
Methotrexate ( 10 25 mg weekly )
Triple antiretroviral theraphy ( HIV associated variant )
Second line
Topical
Glucocorticoids ( medium to high potency )
Vitamin A analogs
Physical
UVA 1 phototheraphy
UVB ( narrowband ) phototheraphy
UVB phototheraphy
Systemic
Azathioprine ( 100 150 mg/day )
Chyclosporine A ( 5 mg/kg/day )
Fumaric -acid esters
TNF- antagonists