Kayoko Matsunaga

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.

A 70-year-old man presented with an area of scarring alopecia measuring 5&times;8 cm with tufts of hair over the parietal to the right occipital region. At the age of 50, the patient had received a ventriculo-peritoneal shunt for hydrocephalus after an aneurysm of his right vertebral artery.<BR>About one year after that operation, he developed a pustule-like eruption in the parietal area where a cerebral ventricle catheter had been placed. Since then he had undergone various kinds of treatment unsuccessfully.<BR>Culture plates grew Staphylococcus aureus. The patient was successfully treated with topical clindamycin, butyric acid propionate betamethazone and oral roxithromycin and has improved.

We report two Japanese patients with Stevens-Johnson syndrome (SJS) who were investigated for HLA gene markers associated with SJS and toxic epidermal necrolysis. Patient 1 was a 53-year-old woman. She developed eruptions in oral provocation tests and was diagnosed with SJS due to allopurinol, clarithromycin, and loxoprofen sodium. Gene analysis revealed HLA-B^&lowast;5801. Patient 2 was a 54-year-old man. He was positive on a carbamazepine patch test and drug-induced lymphocyte stimulation test, and was diagnosed with SJS due to carbamazepine. HLA-B^&lowast;1502 was not detected on gene analysis.