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.
MeSH terms
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Biopsy, Needle
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Bronchiolitis Obliterans / complications*
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Bronchiolitis Obliterans / diagnosis*
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Bronchiolitis Obliterans / drug therapy
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Child
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Disease Progression
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Drug Therapy, Combination
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Fatal Outcome
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Female
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Gold Compounds / therapeutic use
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Humans
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Immunohistochemistry
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Methotrexate / therapeutic use
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Paraneoplastic Syndromes / complications*
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Paraneoplastic Syndromes / diagnosis*
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Paraneoplastic Syndromes / drug therapy
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Pemphigus / complications*
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Pemphigus / drug therapy
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Pemphigus / pathology*
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Prednisone / therapeutic use
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Severity of Illness Index
Substances
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Gold Compounds
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Prednisone
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Methotrexate