title: Fatal paraneoplastic pemphigus after removal of Castleman's disease in a child.
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.
authors.affiliation: Autoimmune Bullous Diseases Research Center, Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran. daneshpj@sina.tums.ac.ir
authors.forename: Maryam
authors.initials: M
authors.lastname: Daneshpazhooh
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Moeineddin
authors.forename: Amin
authors.initials: A
authors.lastname: Kiani
authors.forename: Zahra S
authors.initials: ZS
authors.lastname: Naraghi
authors.forename: Alireza
authors.initials: A
authors.lastname: Firooz
authors.forename: Maryam
authors.initials: M
authors.lastname: Akhyani
authors.forename: Cheyda
authors.initials: C
authors.lastname: Chams-Davatchi
mesh_terms: D001379:Azathioprine / Q000627:therapeutic use; D001989:Bronchiolitis Obliterans / Q000150:complications; D005871:Castleman Disease / Q000188:drug therapy / Q000601:surgery*; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D010392:Pemphigus / Q000188:drug therapy / Q000473:pathology*; D011239:Prednisolone / Q000627:therapeutic use; D012131:Respiratory Insufficiency / Q000209:etiology; D012720:Severity of Illness Index
chemical_list: D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine