Severe cutaneous necrosis associated with type I cryoglobuli
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Cryoglobulins are circulating immunoglobulins that aggregate and precipitate in cold temperatures in vitro, and can be asymptomatic or cause a variety of manifestations in the skin, nerves, and kidneys in vivo.

A previously healthy 58-year-old woman presented to her local hospital with a 1-month history of painful retiform purpura on her bilateral lower extremities that rapidly progressed to extensive gangrenous necrosis with black eschars involving large areas of the body. The patient denied fever, anorexia, arthralgias, or fatigue. On examination, the patient was in no acute distress with normal vital signs. Her skin examination was notable for a retiform purpuric rash with extensive gangrenous necrosis with large black eschars on the bilateral lower extremities, buttocks, posterior upper arms, anterior forearms, and cheeks.

Laboratory testing at her local hospital found multiple positive qualitative cryoglobulin tests, negative cryofibrinogen, low complement C4, and borderline elevated rheumatoid factor. Renal function, liver function, and complete blood counts were normal other than mild anemia of chronic disease thought to be related to her presenting illness. Initial infectious, malignancy, and standard hypercoagulability workups were negative. A skin biopsy was obtained, and the patient was treated with corticosteroids (CS), plasmapheresis, and 1 dose of rituximab before transfer to an academic institution where further testing was obtained.

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