Small Vessel Vasculitis: A Rare Presentation

Small Vessel Vasculitis: A Rare Presentation

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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 Home viral infection and type 3 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 hospital visits.

Skin biopsy and bone marrow biopsy and detection of cryoglobulins clinched the diagnosis.The importance of the case lies Leveler 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.