title: Paraneoplastic pemphigus with bronchiolitis obliterans in a child.
abstract: Paraneoplastic pemphigus (PNP) is a rare blistering autoimmune disease associated with an underlying neoplasm, mucous membrane erosions, and occasionally bronchiolitis obliterans. Most cases have been reported in adults and the number of childhood cases in the current literature is limited. We describe a young patient with PNP who was initially misdiagnosed as having recurrent Stevens-Johnson syndrome. This patient had an underlying inflammatory myofibroblastic tumor and subsequently developed fatal progressive bronchiolitis obliterans.
authors.affiliation: Department of Medicine, University of Arizona College of Medicine, Tucson, Arizona, USA. winnieaz@hotmail.com
authors.forename: Winnie A
authors.initials: WA
authors.lastname: Mar
authors.forename: Robin
authors.initials: R
authors.lastname: Glaesser
authors.forename: Kristin
authors.initials: K
authors.lastname: Struble
authors.forename: Sue
authors.initials: S
authors.lastname: Stephens-Groff
authors.forename: Jerry
authors.initials: J
authors.lastname: Bangert
authors.forename: Ronald C
authors.initials: RC
authors.lastname: Hansen
mesh_terms: D001707:Biopsy, Needle; D001989:Bronchiolitis Obliterans / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D018450:Disease Progression; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D017612:Gold Compounds / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D008727:Methotrexate / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D010392:Pemphigus / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D011241:Prednisone / Q000627:therapeutic use; D012720:Severity of Illness Index
chemical_list: D017612:Gold Compounds; D011241:Prednisone; D008727:Methotrexate