Nuclear vacuolar acantholytic vesicular dermatitis associated with leukocytoclastic vasculitis.

A 48-year-old obese white woman had had a recurrent vesicular eruption for the previous 5 years. This eruption involved almost all parts of the body, including vaginal mucosa. The plantar and palmar surfaces were not involved. Eruptions start with a prodromal phase: 24 to 48 hours prior to the eruption, the normal-appearing skin becomes painful. A reddish discoloration occurs, and vesicles form within a half hour. The histologic features of the vesicles are unique in that they contain acantholytic cells. The nuclei of these cells are vacuolated and contain a reddish, "target"-appearing substance. The underlying corium shows a necrotizing leukocytoclastic vasculitis. They only effective medication for this disease is cyclophosphamide. Pathogenesis is discussed and is compared with that of other vesiculo-bullous acantholytic lesions with that of leukocytoclastic vasculitis. To the best of our knowledge, this disease appears to be unique.