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- Exfoliative dermatitis (ED), or erythroderma, is a rare disorder characterized by generalized redness and scaling involving >90% of the skin.
- It may develop idiopathically (primary erythroderma) or arise as a generalization of a preceding localized skin disease or reaction to a medication (secondary erythroderma).
- ED may appear suddenly or gradually, occasionally accompanied by fever, chills, and lymphadenopathy.
- When fulminant, ED is potentially life-threatening.
- Cutaneous involvement consists of redness and/or scaling of most of the skin (>90%).
- System(s) affected: skin/exocrine
- Synonym(s): erythroderma; exfoliative erythroderma; red man syndrome (l’homme rouge)
- In the United States: rare; estimated 1% of hospitalizations for skin disease
- Predominant age: >40 years, except when it results from hereditary ichthyoses, or immunodeficiencies, which are most common in the pediatric age group
- Predominant sex: male > female (2 to 4:1)
- No racial predilection
Etiology and Pathophysiology
- Arises idiopathically in up to 20–30% of cases. It can also arise secondary to an underlying cutaneous or systemic disease (see the following text) or as a reaction to medications.
- The mechanisms behind generalization of preexisting skin disease is not well understood. In erythrodermic skin, keratinocytes exhibit an increased mitotic rate and increased transit time through the epidermis.
- Underlying diseases, as noted in the following text
- Certain medications, as noted in the following text
- Male sex
- Age >40 years
Commonly Associated Conditions
- Most commonly associated conditions or diseases reported that present with/develop into ED include the following:
- Psoriasis (most common cause in adults)
- Drug reaction (most common cause in children)
- Atopic dermatitis
- Cutaneous T-cell lymphoma (CTCL, mycosis fungoides)
- Idiopathic in up to 20–30% of cases
- Less commonly, ED has been noted as a finding in the following skin disorders:
- Allergic contact dermatitis
- Stasis dermatitis with secondary autoeczematization
- Pityriasis rubra pilaris (a rare disorder of keratinization)
- Graft-versus-host disease
- Seborrheic dermatitis (Leiner disease) in infants
- Ichthyosiform dermatoses
- Pemphigus foliaceus
- Papulosquamous dermatitis of AIDS
- Fungal disease with id reaction
- Other rare reported associations include the following:
- Reiter syndrome
- Systemic lupus erythematosus
- Hailey-Hailey disease
- Norwegian scabies
- Lichen planus
- Medications: may occur as a reaction to the following drugs: allopurinol, antimalarials, aspirin, barbiturates, captopril, codeine, cefoxitin, cimetidine, dapsone, gold salts, hydantoin, isoniazid, lithium, NSAIDs, omeprazole, para-aminosalicylic acid, penicillin, phenylbutazone, phenothiazine, St. John’s wort, sulfonamide, sulfonylurea, thalidomide, and vancomycin
- May occur as a complication or presenting symptom of the following malignancies (paraneoplastic ED):
- Mycosis fungoides (CTCL)
- Sézary syndrome (leukemic variant of mycosis fungoides)
- Hodgkin disease
- Non-Hodgkin lymphoma and leukemia