Pernio is most frequent in young and middle-aged women and in children. Most cases resolve within 2-3 weeks. Elicit a history of cold exposure or repeated episodes of cold exposure. Cutaneous lesions present 12-24 hours after cold exposure as red or violaceous macules, papules, nodules, or plaques, which may form vesicles or ulcerate. They occur on acral areas, are associated with burning or pruritus, and last 1-3 weeks.
Histology is characterized by an intense perivascular infiltrate extending into subcutaneous fat.
Histologic and immunofluorescent evidence of lupus is present in the skin lesions. CBC and sedimentation rate should be obtained to rule out associated leukemia. Review of patients presenting with pernio shows an increased incidence of antiphospholipid antibody syndrome. Cryoglobulins, cryofibrinogen, and cold agglutinins generally are absent but should be considered as part of the laboratory evaluation in a patient with chronic pernio. Pernio lesions can occur in the setting of lupus erythematosus.