Essential cryoglobulinaemia: a clinical course lasting 25 years

The clinical course of a patient is described whose primary manifestations of a circulating cryo IgGK were cutaneous symptoms of pain, vascular purpura and necrotic ulcerations, especially of the lower extremities. A biopsy specimen of skin lesions showed vasculitis and immune reactants in the vessel walls. The aetiology was not recognized. The effect of primary long-term therapy with prednisone was not essential. Combined therapy with cyclophosphamide and plasmapheresis allowed a satisfactory initial remission but had to be maintained repeatedly in order to reduce the periodic recurrence of clinical signs and symptoms. Renal failure terminated the clinical course at 25 years from onset of symptoms.