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A case of recurrent acute febrile neutrophilic dermatosis in a patient with idiopathic cytopenia of undetermined significance

A 62-year-old woman presented with a 3-day history of painful skin lesions with a high-grade fever. She had no history of alcoholism. On physical examination, red papules, pustules and erythematous plaques were observed on the left arm and axilla, and the right leg (Figure 1). Laboratory tests showed increased white blood cell counts (11,400/μl, with 70.2% neutrophils) and C-reactive protein (19.68mg/dl). Macrocytic anemia was observed (hemoglobin, 9.5mg/dl; mean corpuscular volume, 108.5fl). The results of free-T3, free-T4, thyroid stimulating hormone were normal. A biopsy from the left arm showed intraepidermal pustules, as well as a dense and diffuse neutrophilic dermal infiltrate with edema (Figure 2a, b). Because these findings were compatible with Sweet's syndrome (SS), oral prednisolone (30mg/day) was initiated, resulting in a rapid resolution of the lesions.