Abstract
Pemphigus vulgaris is a disease presenting with various-sized blisters. It is known that the titer of anti-intercellular antibody correlates closely with clinical findings. We experienced a case of pemphigus vulgaris who had multiple organ failure in whom treatment with double filtration plasmapheresis (DFPP) was effective.
A 57-year-old woman was admitted because of generalized blisters and erosions. On admission, she had oliguria, azotemia, liver dysfunction, and skin infection due to staphylococcus sp. and pseudomonas sp. The antibody titer was very high, being 104.
We initiated respiratory support with a respirator, frequent hemodialysis and blood transfusion. Administration of antibiotics and high doses of corticosteroids was done simultaneously. Despite this aggressive treatment, the skin lesions did not improve and sepsis developed. We had to reduce the corticosteroid dosage and performed 6 courses of DFPP to reduce antibody levels. With treatment, the antibody titers gradually decreased and improvement of her general condition was observed.
This case shows that DFPP, in combination with corticosteroids, reduces circulating antibody levels more rapidly than conventional therapy and is effective treatment for pemphigus vulgaris without the use of high dose corticosteroids.
