Acute febrile neutrophilic dermatosis

A 68-year-old woman presents with a 6-week history of tender lesions of the palms. Currently, she is undergoing chemotherapy for acute myelogenous leukemia.

On examination there is a symmetrical pattern of tender, red, indurated plaques on the palms. The lesions are edematous, but there is no vesiculation. (Figure)

Differential diagnosis

Conditions to consider are those which produce indurated inflammatory plaques.

Erythema multiforme produces multiple red lesions on the palms, which are occasionally tender. The papules often have a target-like appearance and there are almost always lesions elsewhere on the skin.

Secondary syphilis can have red lesions on the palms. There is scale on the surface and there are always similar papules elsewhere on the skin.

Fixed drug eruption has one or a few red, indurated papules and plaques, associated with ingestion of one of many medications, including cancer chemotherapy agents. These lesions usually have a violaceous center, often with vesiculation.

Contact dermatitis can cause inflammatory lesions on the palms. However, the lack of blistering and pruritus is against this diagnosis.

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is the correct diagnosis. This is a hypersensitivity reaction associated with leukemia, inflammatory bowel disease, and certain infections. It may herald the onset of the underlying disease process or signal a flare-up of a pre-existing condition. It is often accompanied by an elevated white blood cell count.

If Sweet's syndrome occurs without a known cause, a work-up is indicated for underlying leukemia, inflammatory bowel disease, or other inflammatory disorders.

In this case, the patient was treated with prednisone 1 mg per kg/d for 3 weeks, which cleared the eruption completely. However, it should be noted that the successful management of Sweet's syndrome with systemic corticosteroids has no effect on the ultimate outcome of the underlying disease process.

Diagnostic pearl

Consider Sweet's syndrome in a patient with tender red papules, plaques or nodules, especially if this occurs with an elevated white blood cell count.

Dr. Levine is professor of medicine (dermatology), University of Arizona Health Sciences Center, Tucson.

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