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Home :: Exfoliative Erythroderma Syndrome

Exfoliative Erythroderma Syndrome

The exfoliative erythroderma syndrome (EES) is a serious, at times life-threatening reaction pattern of the skin characterized by generalized and uniform redness and scaling involving practically the entire skin and associated with systemic "toxicity," generalized lymphadenopathy, and fever. Two stages, acute and chronic, merge one into the other. In the acute and subacute phases, there is rapid onset of generalized vivid red erythema and fine branny scales; the patient feels hot and cold, shivers, and has fever. In the chronic EES, the skin thickens, and scaling continues and becomes lamellar. There is a loss of scalp and body hair, the nails become thickened and separated from the nail bed (onycholysis), and there may be hyperpigmentation or patchy loss of pigment in patients whose normal skin color is brown or black. About 50% of the patients with EES have a history of a preexisting dermatosis, which is recognizable only in the acute or subacute stages. The most frequent preexisting skin disorders are (in order of frequency) psoriasis, eczematous dermatitis (atopic, allergic contact), adverse cutaneous drug reaction, lymphoma, and pityriasis rubra pilaris. Drugs most commonly implicated in erythroderma. In 10 to 20% of patients it is not possible to identify the cause by history or histology.

Causes of Exfoliative Erythroderma Syndrome

The metabolic response to exfoliative dermatitis may be profound. Large amounts of warm blood are present in the skin due to the dilatation of capillaries, and there is considerable heat dissipation through insensible fluid loss and by convection. Also, there may be high output cardiac failure; the loss of scales through exfoliation can be considerable, up to 9 g/m² of body surface per day, and this may contribute to the reduction in serum albumin and the edema of the lower extremities so often noted in these patients.

Symptoms of Exfoliative Erythroderma Syndrome

Skin Lesions Skin is red, thickened, scaly. Dermatitis is uniform involving the entire body surface, except for pityriasis rubra pilaris, where EES spares sharply defined areas of normal skin.

Hair Thinning of hair, even alopecia, except for EES arising in eczema or psoriasis.

Diagnosis

Diagnosis is not easy, and the history of the pre-existing dermatosis may be the only clue. Also pathognomonic signs and symptoms of the pre-existing dermatosis may help, e.g., dusky-red color in psoriasis and yellowish-red in pityriasis rubra pilaris; typical nail changes of psoriasis; lichenification, erosions, and excoriations in atopic dermatitis and eczema; diffuse, relatively nonscaling palmar hyperkeratoses with fissures in CTCL and pityriasis rubra pilaris; sharply demarcated patches of noninvolved skin within the erythroderma in pityriasis rubra pilaris; massive hyperkeratotic scale of scalp, usually without hair loss in psoriasis and with hair loss in CTCL and pityriasis rubra pilaris; in the latter and in CTCL, ectropion may occur.

Treatment

This is an important medical problem that should be dealt with in a modern inpatient dermatology facility with experienced personnel. The patient should be hospitalized in a single room, at least for the beginning workup and during the development of a therapeutic program. The hospital room conditions (heat and cold) should be adjusted to the patient's needs; most often these patients need a warm room with many blankets.

Topical Water baths with added bath oils, followed by application of bland emollients.

Systemic Oral glucocorticoids for remission induction and for maintenance (except in psoriatic EES); systemic and topical therapy as required by underlying condition.

Supportive Supportive cardiac, fluid, electrolyte, protein replacement therapy as required.

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