Small Vessel Vasculitis: A Rare Presentation

Mixed Cryoglobulinemia (MC) is a rare disorder and often causes diagnostic dilemma.Cryoglobulinemia is of 3 types according to Brouet Classification.Types 2 and 3 are known as MC as type 2 is a mixture of polyclonal IgG and monoclonal IgM associated with viral infection and type 3 Super caps consists of polyclonal IgG, associated with systemic lupus erythematosus.Cryoglobulins must be differentiated from cryofibrinogen which precipitates only in plasma and not in serum.Immune complex deposition leads to cryoglobulinemic vasculitis.

Females are more commonly affected that males.The case presented here, had recurrent episodes of anemia, painful rashes, pedal swelling with subsequent renal dysfunction which ultimately responded to intravenous steroids and rituximab.Hepatitis C Virus infection was ruled out twice during the Mastectomy hospital visits.Skin biopsy and bone marrow biopsy and detection of cryoglobulins clinched the diagnosis.The importance of the case lies in the fact that MC, though a rare disorder, should not be missed in the diagnosis and it should be remembered that HCV negative cryoglobulinemic vasculitis has poor prognosis and may be refractory to treatment.

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