A case of Henoch-Schönlein purpura accompanied by severe gastrointestinal symptoms treated by plasmapheresis

A 52-year-old Japanese man presented at our hospital with low-grade fever, swelling on the right side of the chin, and purpura with blood blisters on the four limbs and hip. Based on the high inflammatory reaction shown by laboratory data, he was diagnosed as having Henoch-Schönlein purpura caused by facial cellulitis, and the pathological examination demonstrated leukocytoclastic vasculitis. Antibiotics were effective for cellulitis, but new purpura developed ; therefore, we started prednisolone (20mg/day), which improved his skin condition. Thereafter, he complained of abdominal pain and hematochezia. Because factor XIII activity was 72%, we administered factor XIII concentrate and increased the prednisolone dose to 60mg/day. There was no obvious discharge, so we decreased prednisolone to 40mg/day. He suddenly developed a massive hemorrhage, causing hemorrhagic shock. Pulsed steroid therapy was not effective, but plasmapheresis markedly improved his abdominal condition. Plasmapheresis is a useful tool to treat severe gastrointestinal symptoms of a non-responder to steroid therapy.