A distinctive variant of pernio. Clinical and histopathologic study of nine cases

Abstract

Nine women are described who had similar distinctive clinical features that consisted of painful red-to-purple macules, papules, and plaques on the digits, predominantly on the toes. All patients reported that exposure to cold had preceded their symptoms. Histologic study demonstrated a lymphocytic vasculitis, characterized by lymphocytes infiltrating blood vessel walls in 8 (88%) of 9 lesions. This inflammation pattern is different from the perivascular lymphocytic infiltrate that is common to lupus erythematosus, erythema multiforme, lymphocytic skin infiltration (Jessner), and drug eruptions. It also serves to distinguish the disorder from other clinical conditions resembling it, such as cellulitis, herpes simplex infection, and disseminated gonococcemia. To the best of our knowledge, this lymphocytic vasculitis was not previously described as occurring in pernio. Apparently the unique disorder reported here is a common, but infrequently recognized, pernio variant.