Failure of double filtration plasmapheresis to treat severe pemphigus vulgaris: A case report.

Abstract

Pemphigus vulgaris (PV) is a chronic, mucocutaneous, autoimmune bullous disease. Double filtration plasmapheresis (DFPP) may be effective when PV fails to be controlled by conventional corticosteroid treatment. The patient was a 64-year-old man with erythema, blisters, and erosions on his head, face, mouth, trunk, limbs, and scrotum for over a month. He was diagnosed with severe PV, and the original rash area continued to expand after treatment with systemic corticosteroids, immunosuppressants, and intravenous immunoglobulin, with massive exudate and ≥5 new blisters and macules still occurring daily. Subsequently, the patient completed three sessions of DFPP. After the first DFPP, the original erosion surface exudate was significantly reduced and gradually healed. After the second DFPP, the erosion area and exudate increased compared with the previous one. After the third DFPP, the rash did not improve further and had a tendency to continue to progress. During the entire three sessions of DFPP, the patient had new blisters and bullae on his limbs every day. The Nikolsky's sign of the limbs turned negative at the initial stage, and then the trunk and limbs Nikolsky's sign became positive again. The titer of autoantibodies did not decrease significantly after the plasmapheresis. The patient eventually died of secondary lung infection and septic shock. The efficacy of DFPP in this patient with refractory severe PV was poor.