Erythroderma (Generalized Exfoliative Dermatitis)

Updated: Apr 14, 2017
  • Author: Sanusi H Umar, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Exfoliative dermatitis (ED) is a definitive term that refers to a scaling erythematous dermatitis involving 90% or more of the cutaneous surface. Exfoliative dermatitis is characterized by erythema and scaling involving the skin's surface and often obscures the primary lesions that are important clues to understanding the evolution of the disease. Clinicians are challenged to find the cause of exfoliative dermatitis by eliciting the history of illness prior to erythema and scaling, by probing with biopsies, and by performing blood studies. See the images below.

Exfoliative dermatitis diffuse skin involvement. Exfoliative dermatitis diffuse skin involvement.
Exfoliative dermatitis close-up view showing eryth Exfoliative dermatitis close-up view showing erythema and scaling.

The term red man syndrome is reserved for idiopathic exfoliative dermatitis, in which no primary cause can be found, despite serial examinations and tests. Idiopathic exfoliative dermatitis is characterized by marked palmoplantar keratoderma, dermatopathic lymphadenopathy, and a raised level of serum immunoglobulin E (IgE) and is more likely to persist than other types.

The term l'homme rouge refers to exfoliative dermatitis that is secondary to cutaneous T-cell lymphoma. The historic classification of exfoliative dermatitis into Wilson-Brocq (a chronic process associated with exacerbation and remissions), Hebra or pityriasis rubra (relentlessly progressive disease), and Savill (self-limiting) types lacks any clinical significance.

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Pathophysiology

An increased skin blood perfusion occurs in exfoliative dermatitis (ED) that results in temperature dysregulation (resulting in heat loss and hypothermia) and possible high-output cardiac failure. The basal metabolic rate rises to compensate for the resultant heat loss. Fluid loss by transpiration is increased in proportion to the basal metabolic rate. The situation is similar to that observed in patients following burns (negative nitrogen balance characterized by edema, hypoalbuminemia, loss of muscle mass).

A marked loss of exfoliated scales occurs that may reach 20-30 g/d. This contributes to the hypoalbuminemia commonly observed in exfoliative dermatitis. Hypoalbuminemia results, in part, from decreased synthesis or increased metabolism of albumin. Edema is a frequent finding, probably resulting from fluid shift into the extracellular spaces. Immune responses may be altered, as evidenced by increased gamma-globulins, increased serum IgE in some cases, eosinophil infiltration, and CD4+ T-cell lymphocytopenia in the absence of HIV infection. Oxidative stress is also associated with drug-induced erythroderma. [1]

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Epidemiology

Race

No racial predilection is reported for exfoliative dermatitis (ED).

Sex

Male-to-female ratio is 2-4:1.

Age

Exfoliative dermatitis onset usually occurs in persons older than 40 years, except when the condition results from atopic dermatitis, seborrheic dermatitis, staphylococcal scalded skin syndrome, or a hereditary ichthyosis. Age of onset primarily is related to etiology. [2, 3]

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Prognosis

The prognosis of exfoliative dermatitis depends largely on underlying etiology.

The disease course is rapid if it results from drug allergy, lymphoma, leukemia, contact allergens, or staphylococcal scalded skin syndrome.

A study35 of pediatric patients (aged <19 y) found that fever is a poor prognostic marker and may indicate a susceptibility to rapid deterioration. In this group, those with the following characteristics have a higher tendency to develop hypotension: age 3 years or younger, ill appearance, vomiting, glucose level of 110 mg/dL or less, calcium value of 8.6 mg/dL or less, platelet count of 300,000/μL or less, elevated creatinine value, polymorphonuclear leukocyte count of 80% or greater, and the presence of a focal infection. The risk of toxic shock syndrome is increased especially in children with erythroderma and fever who have the following additional features: age of 3 years or younger, ill appearance, elevated creatinine value, and hypotension upon arrival.

The disease course is gradual if it results from generalized spread of a primary skin disease (eg, psoriasis, atopic dermatitis).

The mean duration of illness typically is 5 years, with a median of 10 months.

Mortality varies according to the disease's cause. In a study of 91 of 102 patients with exfoliative dermatitis by Sigurdsson et al, [4] a mortality rate of 43% was observed. Only 18% of the deaths were directly related to exfoliative dermatitis. In 74% of the deaths, causes unrelated to exfoliative dermatitis were implicated.

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Patient Education

Educate patients on the specifics of the underlying cause of their exfoliative dermatitis (ED) and the importance of diligent follow-up management as indicated. Patients should be educated on the benefits of a healthy lifestyle and to immediately treat occurrences of erythroderma to better manage their diseases in the long term. Patients should be advised to avoid the use of and/or contact with of irritant soaps, lotions, detergents, and chlorine, and special considerations should be made for allergies, especially for patients with atopic dermatitis. [5]  Excessive sweating should also be avoided.

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