Pemphigus is a rare, blistering autoimmune disease in which the action of antibodies is now well established. The most effective therapy is the administration of corticosteroids. Prior to the use of steroids, it was commonly fatal. Since the
availability of corticosteroids, mortality has been markedly reduced. However prolonged administration of steroids may be difficult to sustain because of severe side effects. For these, other adjuvant therapies were considered. Such as
immunosuppresive
drugs, gold, dapsone, plasmapheresis and intralesional injection of corticosteroids. Of these, immunosuppressive therapies are most commonly used. Recently cyclosporine which was used for human organ transplantaion is used in dermatology.
Cyclosporine
is a potent immunosuppressive agent with no appreciable effect on bone marrow and a selective inhibition to helper T cells. In our case, cyclosporine was administrated to the patient who had suffered from sever pemphigus vulgaris for 3 years
without
responding to treatment. We gave cyclosporine 400mg and prednisolone 90mg per day. But we found a sudden increase of the BUN/Cr level and a shut down of the renal function. Therefore we discontinued cyclosporine and treated acute renal failure.
However
the renal damage was irreversible and two days later the patient died.
(Kor J Dermatal 1994 ; 32(4) : 713~717)
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