Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature.

In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis.

Leg ulcers in active lepromatous leprosy associated with cryoglobulinaemia.

A 40-year-old male agricultural labourer presented with active lepromatous leprosy and painful leg ulcers of 2 months' duration. Biopsy from the ulcer showed nonspecific changes. Raised erythrocyte sedimentation rate and positive rheumatoid factor made us suspect underlying cryoglobulinaemia. Presence of cryoprecipitate in the serum, demonstration of cryoglobulins by serum electrophoresis and raised cryocrit were compatible with cryoglobulinaemia as the cause of atypical leg ulcers in this case. The ulcers healed with bed rest, aspirin and specific anti-leprosy treatment. Though 95% of lepromatous leprosy patients can have cryoglobulinaemia, the presence of atypical ulcers as seen in our patient has not previously been related to the presence of cryoglobulinaemia.

Leg ulcers in active lepromatous leprosy associated with cryoglobulinaemia

A 40-year-old male agricultural labourer presented with active lepromatous leprosy and painful leg ulcers of 2 months' duration. Biopsy from the ulcer showed nonspecific changes. Raised erythrocyte sedimentation rate and positive rheumatoid factor made us suspect underlying cryoglobulinaemia. Presence of cryoprecipitate in the serum, demonstration of cryoglobulins by serum electrophoresis and raised cryocrit were compatible with cryoglobulinaemia as the cause of atypical leg ulcers in this case. The ulcers healed with bed rest, aspirin and specific anti-leprosy treatment. Though 95 per cent of lepromatous leprosy patients can have cryoglobulinaemia, the presence of atypical ulcers as seen in our patient has not previously been related to the presence of cryoglobulinaemia.