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Paraneoplastic Pemphigus with Bronchiolitis Obliterans in a Child
Author(s) -
Mar Winnie A.,
Glaesser Robin,
Struble Kristin,
StephensGroff Sue,
Bangert Jerry,
Hansen Ronald C.
Publication year - 2003
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.2003.20311.x
Subject(s) - bronchiolitis obliterans , medicine , paraneoplastic pemphigus , bronchiolitis , dermatology , pemphigus , mucous membrane , disease , pathology , immunology , lung , respiratory system , autoantibody , lung transplantation , antibody
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.