Erythroderma Summary

Definition

  • Intense widespread reddening of skin
  • Often associated with exfoliation
  • 30% cases idiopathic ("red man syndrome")

Epidemiology

  • Rare condition
  • 3 times more common in males
  • Any age, all races
  • Most have pre-existing condition

Common Causes

  • Drug eruption
  • Dermatitis (especially atopic)
  • Psoriasis (after steroid withdrawal)
  • Pityriasis rubra pilaris

Clinical Features

  • ≥90% skin surface erythema
  • Warm skin, intense itching
  • Scaling after 2-6 days
  • Nail and hair changes

Complications

  • Hypothermia from heat loss
  • Dehydration and electrolyte abnormalities
  • High-output heart failure
  • Infections

Management

  • Often requires hospitalization
  • Discontinue unnecessary medications
  • Fluid/electrolyte balance
  • Skin moisture maintenance

Erythroderma

Intense and widespread reddening of the skin due to inflammatory skin disease

Introduction

Erythroderma is the term used to describe intense and usually widespread reddening of the skin due to inflammatory skin disease. It often precedes or is associated with exfoliation (skin peeling off in scales or layers), when it may also be known as exfoliative dermatitis (ED).

Idiopathic Erythroderma

Idiopathic erythroderma is sometimes called the 'red man syndrome'. About 30% of cases of erythroderma are idiopathic.

Epidemiology & Causes

Epidemiology

  • Rare condition
  • Can arise at any age and in people of all races
  • 3 times more common in males than females
  • Most have pre-existing skin disease or systemic condition

Common Causes

Most Common Skin Conditions

  • Drug eruption (numerous diverse drugs implicated)
  • Dermatitis (especially atopic dermatitis)
  • Psoriasis (especially after withdrawal of systemic steroids)
  • Pityriasis rubra pilaris

Less Common Skin Diseases

  • Other forms of dermatitis (contact, stasis)
  • Blistering diseases (pemphigus, bullous pemphigoid)
  • Sezary syndrome (cutaneous T-cell lymphoma)
  • Rare congenital ichthyotic conditions

Systemic Disease Associations

Malignancies

  • Haematological malignancies (lymphoma, leukaemia)
  • Internal malignancies (rectal, lung, colon, prostate cancer)
  • Paraneoplastic erythroderma

Other Systemic Conditions

  • Graft-versus-host disease
  • HIV infection

Clinical Features

Erythroderma is often preceded by a morbilliform (measles-like) eruption, dermatitis, or plaque psoriasis. Generalised erythema can develop quite rapidly in acute erythroderma, or more gradually over weeks to months in chronic erythroderma.

Signs and Symptoms

Skin Manifestations

  • Generalized erythema and edema affecting ≥90% of skin surface
  • Skin feels warm to touch
  • Troublesome itch, sometimes intolerable
  • Scaling begins 2-6 days after erythema onset
  • Thick scaling on scalp with possible hair loss
  • Yellowish, diffuse keratoderma on palms and soles
  • Nail changes: dull, ridged, thickened, onycholysis

Systemic Features

  • Swollen lymph nodes (dermatopathic lymphadenopathy)
  • Eyelid swelling may result in ectropion
  • Lymphadenopathy, hepatosplenomegaly
  • Abnormal liver dysfunction and fever
  • Leg edema (inflamed skin, heart failure, hypoalbuminaemia)

Complications

Serious Complications

Erythroderma often results in acute and chronic local and systemic complications. The patient is unwell with fever, temperature dysregulation and losing a great deal of fluid by transpiration through the skin.

Systemic Complications

  • Heat loss leading to hypothermia
  • Fluid loss leading to electrolyte abnormalities and dehydration
  • High-output heart failure from red skin
  • Hypoalbuminaemia from protein loss and increased metabolic rate
  • General unwellness leading to pneumonia

Skin Complications

  • Secondary skin infection (impetigo, cellulitis)
  • Longstanding erythroderma may result in pigmentary changes
  • Brown and/or white skin patches

Investigations

Blood Tests

  • Blood count: anemia, white cell abnormalities, eosinophilia
  • Marked eosinophilia should raise suspicions for lymphoma
  • C-reactive protein (may or may not be elevated)
  • Proteins: hypoalbuminaemia and abnormal liver function
  • Polyclonal gamma globulins common
  • Raised IgE typical of idiopathic erythroderma

Skin Investigations

  • Skin biopsies from several sites if cause unknown
  • Nonspecific inflammation on histopathology
  • Diagnostic features may be present
  • Direct immunofluorescence if autoimmune blistering disease suspected

Management

Hospitalization Often Required

Erythroderma is potentially serious, even life-threatening, and the patient may require hospitalisation for monitoring and to restore fluid and electrolyte balance, circulatory status and body temperature.

General Measures

Discontinue all unnecessary medications

Monitor fluid balance and body temperature

Maintain skin moisture with wet wraps, wet dressings, emollients and mild topical steroids

Prescribe antibiotics for bacterial infection

Consider antihistamines for itch (may or may not be helpful)

Specific Treatments

Cause-Specific Management

If a cause can be identified then specific treatment should be started:

  • Topical and systemic steroids for atopic dermatitis
  • Acitretin or methotrexate for psoriasis
  • Appropriate treatment for underlying malignancy if present
  • Treatment of identified drug reactions