ESSENTIALS OF DIAGNOSIS
Desquamation and erythema over most of the body.
Itching, malaise, fever, chills, weight loss.
Erythroderma describes generalized redness and desquamation of more than 30% of the skin surface. A preexisting dermatosis is the cause of exfoliative dermatitis in two-thirds of cases, including psoriasis, atopic dermatitis, contact dermatitis, pityriasis rubra pilaris, and seborrheic dermatitis. Reactions to topical or systemic medications account for about 15% of cases, cancer (paraneoplastic symptom of lymphoma, solid tumors, and most commonly, cutaneous T-cell lymphoma) for 10%, and 10% are idiopathic. Widespread scabies is an important consideration since patients with erythrodermic presentation are highly contagious. At the time of acute presentation, without a clear-cut prior history of skin disease or medication exposure, it may be impossible to make a specific diagnosis of the underlying condition, and diagnosis may require continued observation.
Symptoms may include itching, weakness, malaise, fever, and weight loss. Chills are prominent. Erythema and desquamation are widespread. Loss of hair and nails can occur. Generalized lymphadenopathy may be due to lymphoma or leukemia or may be reactive. The mucosae are typically spared.
A skin biopsy is required and may show changes of a specific inflammatory dermatitis or cutaneous T-cell lymphoma. Peripheral leukocytes may show clonal rearrangements of the T-cell receptor in Sézary syndrome.
Protein and electrolyte loss as well as dehydration may develop in patients with generalized inflammatory exfoliative erythroderma; sepsis may occur.
Home treatment is with cool to tepid baths and application of mid-potency corticosteroids under wet dressings or with occlusion with plastic wrap. If the condition becomes unmanageable in an outpatient setting, the patient should be hospitalized.
Stop all medications, if possible. Systemic corticosteroids may provide marked improvement in severe or fulminant exfoliative dermatitis but should be avoided long-term (see Chapter 26). For cases of psoriatic erythroderma and pityriasis rubra pilaris, acitretin, methotrexate, cyclosporine, or a TNF inhibitor may be indicated. Erythroderma secondary to lymphoma or leukemia requires specific topical or systemic chemotherapy. Suitable antibiotic medications with coverage for Staphylococcus should be given when there is evidence of bacterial infection.
Careful follow-up is necessary because identifying the cause of exfoliative erythroderma early in the course of the disease may be impossible. Most patients improve or recover completely but some require long-term therapy. Deaths are rare in the absence of cutaneous T-cell lymphoma. A minority of patients will suffer from undiminished erythroderma for indefinite periods.