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CASE REPORT
Since 1996 a 32 year old woman had had cold-induced clinical manifestations: Raynaud’s phenomenon, livedo reticularis, necrotic and purpuric lesions on the legs, and acrocyanosis on the ears and fingers. She had no family history of cold intolerance, autoimmune disease, or recurrent thrombosis. In 1998 these symptoms were traced to a monoclonal IgG1λ cryoglobulin (type I) present at 1.5 g/l and precipitating at 27°C. Cold agglutinin and cryofibrinogen were absent while total complement (CH50) and C4 fraction were low. There were no antinuclear, anti-dsDNA, antineutrophil, anticardiolipin, or anti-β2-glycoprotein I antibodies, and viral serology was negative for hepatitis B and C and cytomegalovirus. A skin biopsy showed leucocytoclastic vasculitis and thrombosis of the capillaries related to the cryoglobulin. There were no renal, gastrointestinal, or neurological manifestations, but the patient had intermittent distal polyarthritis and was treated with pentoxifylline accompanied by protective measures against cold.
This mother had born a first healthy child in …