Erythroderma

What is erythroderma?

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). It is sometimes called the ‘red man syndrome’ when no primary cause can be found (idiopathic erythroderma).

Erythroderma

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What causes erythroderma?

Erythroderma can arise from a variety of causes, most often as an extension of a pre-existing skin disorder. Erythroderma may also be due to an adverse drug reaction. However, in as many as 30% of all cases of erythroderma, no underlying cause can be found. This is called idiopathic erythroderma.

The most common pre-existing dermatoses to result in erythroderma include:

• Dermatitis especially atopic dermatitis, contact dermatitis (allergic or irritant) and stasis dermatitis (gravitational eczema) and in babies, seborrhoeic dermatitis,
• Psoriasis
• Pityriasis rubra pilaris
• Blistering diseases including pemphigus and bullous pemphigoid
• Cutaneous T-cell lymphoma (Sezary syndrome)

Erythroderma may also be a symptom or sign of a systemic disease. These may include:

• Internal malignancies eg carcinoma of rectum, lung, fallopian tubes, colon
• Haematological malignancies eg lymphoma, leukaemia
• Graft vs Host disease
• HIV infection

Click here to open a table with list of drugs that have been reported to cause erythroderma. If a drug is the cause, erythroderma is usually preceded by a morbilliform (measles-like) eruption.

What are the clinical features?

Erythroderma that is not due to eczema usually occurs in people older than 40 years. It is slightly more common in men than women. It can develop quite rapidly. Signs and symptoms of erythroderma include:

• Generalised skin redness (erythema) and swelling (oedema) involving 90% or more of the skin surface
• Serous ooze, resulting in clothes and dressings sticking to the skin and an unpleasant smell
• Scaling 2-6 days after the onset of erythema, as fine flakes or large sheets
• Varying degrees of itching, sometimes intolerable
• Thick scaling may develop on scalp with varying degrees of hair loss including complete baldness
• Thickening of palms of hands and soles of feet (keratoderma)
• Eyelid swelling may result in ectropion (exposure of the inside surface of the lower eyelid)
• Nails become ridged and thickened or may shed
• Longstanding erythroderma may result in pigmentary changes (brown and / or white skin patches)
• Secondary infection may occur with pustules and crusting
• Swollen lymph nodes (lymphadenopathy)
• Abnormal temperature control resulting in fever and chills or hypothermia
• Increased heart rate that may result in heart failure in untreated or severe cases particularly in the elderly
• Electrolyte abnormalities and dehydration because of fluid loss through the skin
• Low serum albumin because of protein loss and increased metabolic rate

What is the management of erythroderma?

The underlying cause of erythroderma should be established if possible. Skin biopsies may or may not be helpful. Erythroderma is a serious disease and most patients require hospitalisation to restore fluid and electrolyte balance ,circulatory status and body temperature.

The following general measures apply:

• Discontinue all unnecessary medications
• Monitor fluid balance and body temperature
• Maintain skin moisture with wet wraps, other types of wet dressings, emollients and mild topical steroids
• Antibiotics if secondary infection is present
• Antihistamines for severe itch

If a cause can be identified then specific treatment should be started eg topical steroids for atopic dermatitis; acitretin or methotrexate for psoriasis.

What is the prognosis?

Prognosis of erythroderma depends on the underlying disease process. If the cause can be removed or corrected then prognosis is generally very good. If erythroderma is the result of a generalised spread of a primary skin disorder such as psoriasis or dermatitis, it usually clears with appropriate treatment of the skin disease but may recur at any time. The course of idiopathic erythroderma is unpredictable. It may persist for a long time with periods of acute exacerbation.