2010 Volume 120 Issue 6 Pages 1187-1195
Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by flaccid bullae that become eroded and form vegetations or papillomatous lesions, especially in intertriginous areas. The standard treatment for pemphigus vegetans has been oral administration of corticosteroids. We reported a case of pemphigus vegetans in a 67-year-old Japanese female with a high titer of anti-desmoglein 3 autoantibodies (index value of 14,433.3) treated with double filtration plasmapheresis (DFPP) and pulse cyclophosphamide therapy. The treatment schedule consisted of repeated DFPP with the use of albumin solution as the substitution fluid and subsequent administration of high-dose cyclophosphamide (12 mg/kg; total dose of cyclophosphamide pulse was 600 mg) and 2.5 mg/kg/day of methylprednisolone. The skin lesions were cured after three treatments with DFPP and cyclophosphamide, and the titer of anti-desmoglein 3 autoantibodies decreased to an index value of 26. No significant adverse effects were observed. Afterwards, she was treated with low dose systemic steroid and tacrolimus. DFPP combined with synchronized pulse cyclophosphamide may be a useful treatment regime in the management of pemphigus vegetans.