Paraneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature

Australas J Dermatol. 2015 May;56(2):120-3. doi: 10.1111/ajd.12264. Epub 2014 Nov 4.


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.

Keywords: inflammatory myofibroblastic tumour; paraneoplastic pemphigus.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Female
  • Humans
  • Inflammation / complications
  • Mediastinal Neoplasms / complications
  • Mediastinal Neoplasms / pathology
  • Mediastinal Neoplasms / therapy*
  • Neoplasms, Muscle Tissue / complications
  • Neoplasms, Muscle Tissue / pathology
  • Neoplasms, Muscle Tissue / therapy*
  • Oral Ulcer / etiology
  • Paraneoplastic Syndromes / etiology
  • Paraneoplastic Syndromes / therapy*
  • Pemphigus / etiology
  • Pemphigus / therapy*