Dermatitis, Exfoliative

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Basics

  • 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.

Description

  • 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).

Epidemiology

Incidence
  • 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.

Risk Factors

  • 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
    • Dermatomyositis
    • Malignancy (paraneoplastic ED)
      • Sézary syndrome (leukemic variant of mycosis fungoides)
      • Hodgkin disease
      • Non-Hodgkin lymphoma and leukemia
      • Cancer of lung, colon, prostate, and thyroid

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Basics

  • 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.

Description

  • 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).

Epidemiology

Incidence
  • 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.

Risk Factors

  • 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
    • Dermatomyositis
    • Malignancy (paraneoplastic ED)
      • Sézary syndrome (leukemic variant of mycosis fungoides)
      • Hodgkin disease
      • Non-Hodgkin lymphoma and leukemia
      • Cancer of lung, colon, prostate, and thyroid

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