We report a case of paraneoplastic pemphigus (PNP). The 62-year-old Japanese female showed clinical, histopathological and immunohistopathological features characteristic of PNP. The results of antigen analysis by both immunoblotting and immunoprecipitation were compatible with those of PNP. The patient exhibited a bronchiolitis obliterans-like respiratory symptoms, and the course was progressive and lethal in spite of extensive therapies, including methylpredonisolone pulse therapy, high-dose cyclosporin and plasmapheresis. Extensive examination and an autopsy did not reveal an apparent tumor. This is a rare case in which no tumor was detected.
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