Extensive thrombotic purpura revealing multiple myeloma associated - type I cryoglobulinemia

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Extensive thrombotic purpura revealing multiple myeloma associated – type I cryoglobulinemia

A 69-year-old Caucasian male was hospitalized for recurrent attacks of ‘cutaneous vasculitis’ resistant to oral corticosteroids. On admission, he presented with extensive purpuric necrotic patches of the dorsum of the hands, the lumbar areas and the lower limbs (image). The lesions were sharply demarcated and mildly painful. The face and trunk were spared. The patient also described arthralgia of the knees and heels. Two 4-mm punch skin biopsies were performed in the centre and on the edge of a necrotic plaque. Microscopy showed noninflammatory hyaline thrombosis of the small blood vessels in the upper and deep dermis. There were no signs of cutaneous vasculitis or embolism (bottom right). Direct immunofluorescence (DIF) did not show any immunoglobulin deposits. Laboratory findings revealed an inflammatory syndrome with elevated C-reactive protein [145 mg/l, normal range (NR)