A fatal case of bullous pemphigoid with hemophagocytic syndrome in a patient undergoing 15 years of peritoneal dialysis

We herein report a fatal case of bullous pemphigoid (BP) with hemophagocytic syndrome associated with sepsis due to MRSA. A 62-year-old Japanese woman visited us with oral erosions and pea-sized, itchy erythemas on her trunk. She had a 15-year history of peritoneal dialysis due to chronic renal failure, from which she had been suffering since 22 years of age. Treatment with steroid ointments was started. Two weeks later, she developed bullae on her upper limbs. A diagnosis of BP was made based on the findings of direct immnofluorecence and indirect immunofluorescence studies. Control with systemic prednisolone alone for renal failure and heart failure was difficult, and we thus began to add double filtration plasmapheresis. After 6 trials of double filtration plasmapheresis, the bulla formation stopped, with only a few persisting in the dorsal area of the feet. The erosions in the dorsal area of the feet were determined to be concurrent infections with Pseudomonas aeruginosa and MRSA. At the same time, she was found to be suffering from ITP for thrombocytopenia and anti-platelet assosiated IgG antibody showing a titer of 1126.7ng/10⁷ cells. Sudden bleeding from the rectal ulcer led to shock followed by peritonitis with MRSA and sepsis. An examination of the bone marrow revealed hemophagocytosis, leading to a diagnosis of hemophagocytosys syndrome. Inspite of intensive treatment with systemic and, intraperitoneal antibiotics, steroid pulse and immune serum globulin, she eventually died of pancytopenia and MRSA sepsis.