Small-Vessel Vasculitis

Mixed cryoglobulinemia

Chronic purpura with post-inflammatory dystrophic and pigmentary changes on the leg, small ulcer on the left foot, and incipient gangrene of a toe in a patient with mixed cryoglobulinemia.

Clinical Background:

A 36 year old woman suffering from a mixed cryoglobulinemia for 12 years. Ongoing purpuric eruptions had progressively determined a clinical picture of cutaneous postinflammatory dystrophic and pigmentary changes on the legs.   At the age of 31, several small cutaneous ulcers developed on both legs and the patient was therefore referred to our Department.   Laboratory examinations revealed the presence of a chronic hepatitis C with high levels of bilirubin and aminotransferases, anti-HCV antibodies, decreased complement levels, high titers of rheumatoid factor, cryoimmunoglobulins (cryocrit 80%), proteinuria (1.5 g/d) but no antinuclear autoantibodies or antibodies to extractable nuclear antigens.
Physical examinttion showed an enlarged liver and spleen, as well as hypertension.
She was therefore started on prednisone treatment, colchicine, enalapril and plasma exchange with improvement of the proteinuria and the cutaneous ulcers within four months.
One year later she returned to our Department because of an incipient gangrene of a toe accompanied by an exacerbation of the renal involvement for which she received ILOPROST infusions for 20 days and a second plasma exchange.   Her toe slowly healed however, some months after plasma exchange withdrawal, cutaneous ulcers relapsed and therefore alfa-interferon therapy was started.

All contents copyright of the "On-line Archives of Rheumatology", 1996