title: Paraneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature.
abstract: 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.
authors.affiliation: Autoimmune Bullous Diseases Research Centre, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran; Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Narges
authors.initials: N
authors.lastname: Ghandi
authors.forename: Alireza
authors.initials: A
authors.lastname: Ghanadan
authors.forename: Mohammad-Reza
authors.initials: MR
authors.lastname: Azizian
authors.forename: Pardis
authors.initials: P
authors.lastname: Hejazi
authors.forename: Nessa
authors.initials: N
authors.lastname: Aghazadeh
authors.forename: Parvin
authors.initials: P
authors.lastname: Tavousi
authors.forename: Maryam
authors.initials: M
authors.lastname: Daneshpazhooh
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007249:Inflammation / Q000150:complications; D008479:Mediastinal Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy*; D009379:Neoplasms, Muscle Tissue / Q000150:complications / Q000473:pathology / Q000628:therapy*; D019226:Oral Ulcer / Q000209:etiology; D010257:Paraneoplastic Syndromes / Q000209:etiology / Q000628:therapy*; D010392:Pemphigus / Q000209:etiology / Q000628:therapy*
keywords: inflammatory myofibroblastic tumour; paraneoplastic pemphigus