P araneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature | Zendy

Ghandi Narges | Zendy; Ghanadan Alireza | Zendy; Azizian MohammadReza | Zendy; Hejazi Pardis | Zendy; Aghazadeh Nessa | Zendy; Tavousi Parvin | Zendy; Daneshpazhooh Maryam | Zendy

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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.

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