Exfoliative Dermatitis Erythroderma Syndrome “Skin Failure”
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 (>90% surface) and associated with systemic “toxicity,” generalized lymphadenopathy, and fever. In the acute and sub acute 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, seborrheic), adverse cutaneous drug reaction, lymphoma, and pityriasis rubra pilaris. Drugs most commonly implicated in erythroderma are found In 10 to 20% of patients it is not possible to identify the cause by history or histology.
Pathogenesis
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.
Systemic changes associated with exfoliative dermatitis and erythroderma
i. Hypothermia and hyperthermia
ii. Fluid and electrolyte disturbance
iii. Sepsis
iv. Pyrexia occurs due to pyrogens transcutaneously.
v. Hypoprotienemia from exfoliation
vi. Anemia
vii. Vitamin deficiency states
viii. By unknown mechanism they have they have GIT disorders such as mal absorption.
Possible Etiology of Exfoliative Dermatitis in Adults
- Undetermined or unclassified 23%
- Psoriasis 23%
- Atopic dermatitis, eczema 16%
- Drug allergy 15%
- Lymphoma, leukemia 11%
- Allergic contact dermatitis 5%
- Seborrheic dermatitis 5%
- Stasis dermatitis with “id” reaction 3%
- Pityriasis rubra pilaris 2%
- Pemphigus foliaceus 1%
Physical examination
Appearance of Patient frightened, red, “toxic.” Skin is red, thickened, and scaly. Dermatitis is uniform involving the entire body Surface except for pityriasis rubra pilaris, where EES spares sharply defined areas of normal skin. Thickening leads to exaggerated skin folds; scaling may be fine and branny, and may be barely perceptible or large, up to 5 cm, and lamellar.
Palms and Soles Usually involved, with massive hyperkeratosis and deep fissures in pityriasisrubra pilaris, Sézary’s syndrome, and psoriasis.
Hair - Thinning of hair, even alopecia, except for EES arising in eczema or psoriasis.
Nails - Onycholysis, shedding of nails.
General Examination Lymph nodes generalized, rubbery, and usually small; enlarged in Sézary’s syndrome. Edema of lower legs and ankles.
Diagnosis
Diagnosis is not easy, and the history of the preexisting dermatosis may be the only clue. Also, pathognomonic signs and symptoms of the preexisting 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 scaly eczematous lesions starting form the scalp and seborrheic area may suggest seborrheic eczema.
Course and Prognosis
Prognosis depends on underlying etiology. Despite the best attention to all details, patients may succumb to infections or, if they have cardiac problems, to cardiac failure (“high output” failure) or to the effects of the prolonged glucocorticoid therapy that may be required.
Management
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 cardiac, fluid, electrolyte, protein replacement therapy as required.
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