Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands - A report of 7 cases and review of the literature

Background: Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands is a recently described disorder, which may clinically resemble a localized variant of Sweet syndrome. Objectives: To describe the clinical and histopathologic characteristics of this rare disorder; to compare and contrast these features with those of Sweet syndrome; and to investigate possible associations with systemic diseases. Observations: Seven women were referred for pustular or ulcerative plaques and nodules on the dorsal hands. In most patients, the initial diagnosis was cutaneous infection, but antibiotic therapy was ineffective. Skin biopsy specimens showed dense dermal neutrophilic infiltrates with leukocytoclasis and fibrinoid vascular necrosis. Cutaneous cultures yielded negative findings in all cases. Prednisone and dapsone appeared to be helpful, but recurrences were common. Minocycline hydrochloride was of uncertain benefit. Among the 7 patients, possible systemic associations included bowel disorders and a urinary tract infection. Conclusions: Neutrophilic dermatosis of the dorsal hands may be closely related to Sweet syndrome but frequently shows the histologic pattern of leukocytoclastic vasculitis. Recognition of this disorder is important, because it may be misdiagnosed as a localized cutaneous infection. Additional studies are needed to investigate further the possible associations with internal diseases, especially bowel disorders.