Febrile neutrophilic dermatosis associated with acute leukemia

Febrile neutrophilic dermatosis (Sweet's Syndrome) is an uncommon syndrome characterized clinically by fever, neutrophilia, and erythematous, non-ulcerating papules or plaques on the face, neck, upper thorax, and extremities. A case associated with acute myelogenous leukemia is reported and nine other reported cases associated with acute non-lymphocytic leukemia are reviewed. The appearance and location of the lesions on the face, neck, upper trunk and extremities, a female predominance, an associated fever, marked dermal infiltrates of mature neutrophils, and rapid clearing of lesions with parenteral corticosteroids were manifestations similar to febrile neutrophilic dermatosis not associated with leukemia. Although the etiology of febrile neutrophilic dermatosis associated with acute leukemia is unknown, the resolution of lesions and symptoms with systemic corticosteroids and recurrence with relapse of leukemia suggests an immune process with the leukemic cell population serving as a possible antigenic source.