eMedicine Specialties > Dermatology > Papulosquamous Diseases

Erythroderma (Generalized Exfoliative Dermatitis)

Author: Sanusi H Umar, MD, FAAD, Attending Physician, Department of Dermatology, Charles R Drew/MLK Medical Center
Coauthor(s): A Paul Kelly, MD, Chief, Clinical Professor, Department of Internal Medicine, Division of Dermatology, King/Drew Medical Center, Charles R Drew University
Contributor Information and Disclosures

Updated: Aug 24, 2009

Introduction

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.

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 (chronic process associated with exacerbation and remissions), Hebra or pityriasis rubra (relentlessly progressive disease), and Savill (self-limiting) types lacks any clinical significance.

Pathophysiology

An increased skin blood perfusion occurs in exfoliative dermatitis 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, and CD4⁺ T-cell lymphocytopenia in the absence of HIV infection.

Mortality/Morbidity

Mortality varies according to the disease's cause. In a study of 91 of 102 patients with exfoliative dermatitis by Sigurdsson et al,¹ 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.

Race

No racial predilection is reported for exfoliative dermatitis.

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.

Clinical

History

History is the most important aid in diagnosing exfoliative dermatitis (ED).

• Patients may have a history of the primary disease (eg, psoriasis, atopic dermatitis).
• Elicit a comprehensive drug history, including over-the-counter drugs.
• Disease usually evolves rapidly when it results from drug allergens, lymphoma, leukemia, or staphylococcal scalded skin syndrome.
• Disease evolution is more gradual when it results from psoriasis, atopic dermatitis, or the spread of primary disease.
• Pruritus is a prominent and frequent symptom.
• Malaise, fever, and chills may occur.

Physical

• Patients often present with generalized erythema.
• Scaling appears 2-6 days after the onset of erythema, usually starting from flexural areas.
• Pruritus commonly results in excoriations.
• When exfoliative dermatitis persists for weeks, hair may shed; nails may become ridged and thickened and also may shed.
• Periorbital skin may be inflamed and edematous, resulting in ectropion (with consequent epiphora).
• In chronic cases, pigmentary disturbances can occur (especially in darker-skinned races); patchy or widespread loss of pigment (resembling vitiligo) has been reported.
• Diligent search for residual signs of underlying disease occasionally yields dividends. Residual signs may include the following:
  • Islands of sparing in pityriasis rubra pilaris
  • Few typical psoriatic plaques in psoriasis
  • Papules or oral lesions of lichen planus
  • Superficial blisters of pemphigus foliaceus
  • Erythematous papular lesions of an early drug eruption
• Dermatopathic lymphadenopathy can occur in exfoliative dermatitis not caused by lymphoma or leukemia. A lymph node biopsy is advised when lymph nodes exhibit lymphomatous characteristics (eg, large size, rubbery consistency) and the cause of exfoliative dermatitis is undetermined.
• The general picture is modified according to the nature of the underlying disease and the patient's general physical condition.

Causes

• Atopic dermatitis - Acute and chronic leukemia
• Contact dermatitis - Reticulum cell sarcoma
• Dermatophytosis - Carcinoma of rectum
• Hailey-Hailey disease - Carcinoma of fallopian tubes
• Leiner disease -Graft versus host disease
• Lichen planus - HIV infection
• Lupus erythematosus - Lymphoma (including Hodgkin disease)
• Mycosis fungoides -Multiple myeloma
• Pemphigoid - Carcinoma of lung
• Pemphigus foliaceus - Mycosis fungoides
• Pityriasis rubra pilaris - Reiter syndrome
• Psoriasis
• Sarcoid
• Seborrheic dermatitis
• Stasis dermatitis

The most common causes of exfoliative dermatitis are best remembered by the mnemonic device ID-SCALP. The causes and their frequencies are as follows:

• Idiopathic - 30%
• Drug allergy - 28%
• Seborrheic dermatitis - 2%
• Contact dermatitis - 3%
• Atopic dermatitis - 10%
• Lymphoma and leukemia - 14%
• Psoriasis - 8%

More than 60 drugs have been implicated in the causation of exfoliative dermatitis (see Table). In many cases of protracted exfoliative dermatitis classified as being of undetermined cause, careful follow-up care and reevaluation implicated atopic dermatitis in older patients, intake of drugs overlooked by the patient, and prelymphomatous eruption as causative factors.

Drugs Implicated in the Causation of Exfoliative Dermatitis

Open table in new window

• Search for CME/CE on This Topic »