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- Exfoliative dermatitis (ED), also known as erythroderma, is a rare and severe cutaneous disorder characterized by generalized erythema involving >90% of the skin and variable degree of scaling.
- It may develop idiopathically (primary erythroderma) or secondary to preexisting skin disorders, malignancies, infections, and drugs (secondary erythroderma).
- ED may appear suddenly or gradually, and can be accompanied by fever, chills, malaise, and lymphadenopathy.
- When fulminant, ED is potentially life-threatening.
- ED can be acute or chronic and follow a relapsing and remitting course. Cutaneous involvement consists of widespread redness (>90% of total body surface) and exfoliation (skin peels off).
- 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 to 60 years, except when it results from hereditary ichthyoses or immunodeficiencies, which are most common in the pediatric age group
- Predominant sex: male > female (2:1 to 4:1)
- No racial predilection
Etiology and Pathophysiology
- Idiopathic condition in up to 20–30% of cases. Exacerbation of a preexisting dermatosis is the most common known cause of ED in adults. It can also occur secondary to a systemic disease, drug reaction, infection, or malignancy (see “Commonly Associated Conditions”).
- 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 and certain medications (see “Commonly Associated Conditions”)
- Male sex
- Age >40 years
Commonly Associated Conditions
- Most common associated diseases and medications that present with or develop into ED include the following:
- Psoriasis (most common cause in adults)
- Atopic dermatitis
- Contact (allergic and irritant) dermatitis
- Cutaneous T-cell lymphoma (CTCL, mycosis fungoides)
- Pityriasis rubra pilaris
- Drug reaction (most common cause in children)
- ACE inhibitors, allopurinol, antibiotics (mainly β-lactams and vancomycin), antimalarials, barbiturates, carbamazepine, cimetidine, codeine, dapsone, gold salts, isoniazid, lamotrigine, lithium, NSAIDs, phenothiazine, phenytoin, and proton pump inhibitors and sulfonamides
- Less commonly associated disorders:
- Graft-versus-host disease
- Infections (e.g., HIV, Norwegian scabies)
- Fungal disease with id reaction
- Seborrheic dermatitis
- Ichthyosiform dermatoses
- Bullous disorders (e.g., pemphigus foliaceus)
- Reiter syndrome
- Systemic lupus erythematosus
- Hailey-Hailey disease
- Malignancy (paraneoplastic ED)
- Sézary syndrome (leukemic variant of mycosis fungoides)
- Hodgkin disease
- Non-Hodgkin lymphoma and leukemia
- Cancer of lung, colon, prostate, and thyroid