Oesophageal pemphigoid: a rare cause of dysphagia

Clin J Gastroenterol. 2019 Feb;12(1):25-28. doi: 10.1007/s12328-018-0897-6. Epub 2018 Aug 23.

Abstract

Pemphigus vulgaris (PV) is a rare autoimmune bullous disease which affects the skin and mucous membranes. Oesophageal involvement is rare and has previously been limited to case reports and case series. A recent large case series of 477 PV patients showed that 26/477 (5.4%) had symptomatic oesophageal involvement. We present the case of a 54-year-old Somalian lady with a 10-year history of cutaneous PV, currently in remission, who developed dysphagia and odynophagia and was subsequently found to have oesophageal PV involvement with multiple flaccid bullae which were positive for anti-DSG3 antibodies on in-direct immunofluorescence. She had her treatment switched from azathioprine to mycophenolate and prednisolone, leading to resolution of her symptoms.

Keywords: Dysphagia; Oesophageal; Pemphigus vulgaris.

Publication types

  • Case Reports

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Autoantibodies / analysis
  • Deglutition Disorders / etiology*
  • Desmoglein 3 / immunology
  • Esophageal Diseases / complications*
  • Esophageal Diseases / drug therapy
  • Esophageal Diseases / immunology
  • Female
  • Humans
  • Immunologic Factors / therapeutic use
  • Microscopy, Fluorescence
  • Middle Aged
  • Mycophenolic Acid / therapeutic use
  • Pain / etiology
  • Pemphigus / complications*
  • Pemphigus / drug therapy
  • Pemphigus / immunology
  • Prednisolone / therapeutic use

Substances

  • Anti-Inflammatory Agents
  • Autoantibodies
  • DSG3 protein, human
  • Desmoglein 3
  • Immunologic Factors
  • Prednisolone
  • Mycophenolic Acid