Paraneoplastic Pemphigus Associated With Inflammatory Myofibroblastic Tumour of the Mediastinum: A Favourable Response to Treatment and Review of the Literature Publisher Pubmed



Ghandi N^{1, 2} ; Ghanadan A^{3, 4} ; Azizian MR⁵ ; Hejazi P^{1, 2} ; Aghazadeh N^{1, 2} ; Tavousi P^{1, 2} ; Daneshpazhooh M^{1, 2}
Source: Australasian Journal of Dermatology Published:2015

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. © 2014 The Australasian College of Dermatologists.