Cutaneous manifestations of cryoglobulinemia: clinical and histopathologic study of seventy-two patients.

Abstract

In the 72 cases of cryoglobulinemia reviewed, erythematous to purpuric macules or papules were present in 92%. Infarction, hemorrhagic crusts, and ulcers were present in 10% to 25% of the patients and were relatively more common in type I cryoglobulinemia than in the other types. Postinflammatory hyperpigmentation was noted in 40%. Lesions on the leg were common in all types of cryoglobulinemia; however, lesions on the head and mucosal surfaces suggested type I cryoglobulinemia. Histopathologic features were classified as vasculitis in 50%, inflammatory or noninflammatory purpura in 15%, noninflammatory hyaline thrombosis in 10%, and postinflammatory sequelae in 10%. Noninflammatory hyaline thrombosis was relatively more frequent in type I. Thus erythematous to purpuric lesions on the legs and leukocytoclastic vasculitis are the common cutaneous findings in cryoglobulinemia. Type I cryoglobulinemia is suggested by noninflammatory hyaline thrombosis, cutaneous infarction, hemorrhagic crusts, skin ulcerations, and lesions of the head and neck and of oral or nasal mucosa.