Thalidomide for Type 1 Cryoglobulinemic Vasculopathy

A 46-year-old African American woman was referred to 1 of us (J.P.C.) for evaluation and treatment of a vasculopathy in November 2003. She had been well until October 2003, when, following an upper respiratory tract infection, she developed painful lesions on her calves. She had been treated with a short course of oral prednisone by the referring dermatologist, which resulted in minimal improvement. Review of the pathologic specimen, which was obtained by the referring dermatologist, confirmed a noninflammatory vasculopathy in which there was plugging of multiple small cutaneous vessels with a proteinaceous material. Laboratory evaluation revealed a monoclonal spike on protein electrophoresis and a positive qualitative cryofibrinogen finding. Findings from complete blood cell count and comprehensive chemical analysis were normal, and findings for anticardiolipin antibody, antineutrophilic cytoplasmic antibody, and hepatitis C antibody were negative. Physical examination on referral revealed an obese African American woman with a reticulated pattern of erythematous to violaceous, superficially necrotic, and ulcerated papules and plaques on the posterior aspect of the calves and medial aspect of the thighs. Further testing with immunofixation electrophoresis revealed an IgG monoclonal paraprotein with light-chain specificity of which 98% was a type I cryoglobulin. Analysis of a bone marrow biopsy specimen revealed a clonal plasma cell expansion occupying 5% to 6% of the specimen. A chest radiograph, skeletal survey, and computed tomography scan of the chest and abdomen were unremarkable. She was initially treated with oral mycophenolate mofetil, 1 g twice daily, which was eventually increased to 1.5 g twice daily, but after 3 months of therapy, there was little improvement, and the mycophenolate mofetil treatment was discontinued. In May 2004, she continued to have painful ulcerations on her calves, which were debilitating (Figure 1).