Fatal Paraneoplastic Pemphigus After Removal of Castleman’s Disease in a Child


Address correspondence to Maryam Daneshpazhooh, M.D., Autoimmune Bullous Diseases Research Center, Department of Dermatology; Razi Hospital, Vahdate-Eslami Sq, Tehran 11996, Iran, or e-mail: daneshpj@sina.tums.ac.ir.


Abstract:  Paraneoplastic pemphigus (PNP) is seen most frequently in the setting of Castleman’s disease (CD) in childhood. We report herein a 10-year-old girl with PNP appearing a few weeks after resection of a recurrent CD. Despite improvement in skin and mucosal lesions with prednisolone and azathioprine, she had severe bronchiolitis obliterans and died from respiratory failure a few months later.