Castleman’s disease complicated by bronchiolitis obliterans without any symptoms of paraneoplastic pemphigus: A case report

Abstract

Castleman’s disease of the hyaline-vascular type is sometimes associated with paraneoplastic pemphigus (PNP) and causes bronchiolitis obliterans. We encountered a case in which bronchiolitis obliterans accompanied Castleman’s disease without PNP symptoms. A 15-year-old girl was referred to our hospital because of dyspnea and abdominal tumor. Open biopsy revealed that the tumor was associated with Castleman’s disease of the hyaline-vascular type. Even after the complete resection of the tumor, dyspnea did not improve. We considered that her dyspnea was derived from bronchiolitis obliterans; therefore, we administered steroids and high-dose immunoglobulin. Because of the possible progression to respiratory failure, she was added to the waiting list for lung transplantation. Despite the lack of clinical skin and mucosa features associated with Castleman’s disease, PNP-specific autoantibodies against plakin family proteins were detected in the serum of this patient, and thus, she was considered as having occult PNP.