Background: Several patients were observed with a peculiar cutaneous e
ruption limited to the dorsa of the hands and fingers. Clinically the
lesions had some resemblance to those seen in Sweet's syndrome, but bi
opsy specimens showed severe leukocytoclastic vasculitis. Objective: O
ur purpose was to characterize this eruption clinically and histologic
ally and compare it with previously described diseases. Methods: Six p
atients observed since 1977 are described. Skin biopsy specimens were
obtained. Results: In six women (age, 41 to 79 years) a symmetric erup
tion of papules and plaques limited to the dorsa of the radial sides o
f the hands and first three digits developed. The lesions resembled th
ose of Sweet's syndrome and were associated with fever, sterile cultur
e, blood neutrophil leukocytosis, nonresponse to antibiotic therapy, a
nd rapid response to prednisone. Biopsy specimens showed a severe leuk
ocytoclastic vasculitis. Conclusion: These patients appear to have a d
istinct entity that we have termed pustular vasculitis of the hands.