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P araneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature
Author(s) -
Ghandi Narges,
Ghanadan Alireza,
Azizian MohammadReza,
Hejazi Pardis,
Aghazadeh Nessa,
Tavousi Parvin,
Daneshpazhooh Maryam
Publication year - 2015
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/ajd.12264
Subject(s) - medicine , mediastinum , paraneoplastic pemphigus , azathioprine , sarcoma , pathology , pemphigus , neoplasm , dermatology , antibody , immunology , surgery , disease , autoantibody
Paraneoplastic pemphigus ( PNP ) is an autoimmune blistering disorder that occurs in association with an underlying neoplasm. Inflammatory myofibroblastic tumour ( IMT ) is a rare low‐grade sarcoma of fibroblasts and myofibroblasts associated with inflammatory cells, most commonly occurring in the lung. In this study, a case of PNP associated with IMT of the mediastinum is reported. The patient had a favourable outcome following surgical resection and treatment with a systemic steroid, azathioprine, and i.v. immunoglobulin. The occurrence of PNP with sarcomas, specifically IMT , is noteworthy although it is not well studied in the existing literature.