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. Drug-induced 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