Paraneoplastic pemphigus with bronchiolitis obliterans in a child.
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.
Mar
Winnie A
WA
Department of Medicine, University of Arizona College of Medicine, Tucson, Arizona, USA. winnieaz@hotmail.com
Glaesser
Robin
R
Struble
Kristin
K
Stephens-Groff
Sue
S
Bangert
Jerry
J
Hansen
Ronald C
RC
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
D017612:Gold Compounds; D011241:Prednisone; D008727:Methotrexate