IVIG Produces Sustained Long-Term Clinical Remission in Patients with Epidermolysis Bullosa Acquisita

Specialists at the Center for Blistering Diseases in Boston report on outcomes of administration of intravenous immunoglobulin (IVIG) in 10 patients with severe and widespread epidermolysis bullosa acquisita (EBA) who were nonresponsive to conventional therapy. The four Caucasian males and six females, whose ages ranged from 37 to 75 years (mean 57.4) were treated according to a protocol published in a Consensus Statement for treatment of autoimmune mucocutaneous blistering diseases. The patients received 16 to 31 cycles (mean 23.1) of 2 g/kg/cycle over a period of 30 to 52 months (mean 38.8). Once IVIG was initiated, earlier drugs (prednisone, dapsone and others) were gradually withdrawn over a five- to nine-month period. Thereafter, IVIG was used as monotherapy.

A satisfactory clinical response was observed in all 10 patients. No serious side effects were observed. The follow-up period after discontinuation of IVIG varied from 29 to 123 months (mean 53.9). During this follow-up period, recurrence of disease was not observed in any patient. These data suggest that IVIG can produce a long-term sustained clinical remission in patients with EBA while permitting concomitant therapy to be discontinued, the co-authors concluded.