Pemphigus vulgaris: a rare cause of dysphagia.
Pemphigus vulgaris is a rare autoimmune blistering disease of the skin and mucous membranes. The case reported presented unusually with dyspepsia that was not responsive to protein pump inhibitor (PPI) therapy. This progressed to severe dysphagia and odynophagia. An esophagogastroduodenoscopy showed extensive ulceration of the esophagus, and direct immunofluorescence of an esophageal biopsy showed bright intercellular staining with C3 and IgG, confirming the diagnosis of pemphigus vulgaris. Immunological remission was achieved after a number of courses of pulsed intravenous methylprednisolone and cyclophosphamide. The patient has remained in remission for 5 years, but has required regular dilation of esophageal strictures for symptom relief. During this period, a chronic lymphocytosis was incidentally noted on routine blood tests, and chronic lymphocytic leukaemia was diagnosed. It is essential to investigate PPI-resistant symptoms, dysphagia and odynophagia, as they may indicate a serious underlying cause.
East and North Hertfordshire NHS Trust, Stevenage, UK.
Ali
http://orcid.org/0000-0002-2094-0556
A
Al-Janabi
Lister Hospital, Stevenage, UK.
Simon
S
Greenfield
D000368:Aged; D003520:Cyclophosphamide / Q000627:therapeutic use; D003680:Deglutition Disorders / Q000209:etiology*; D016145:Endoscopy, Digestive System; D004935:Esophageal Diseases / Q000209:etiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D010392:Pemphigus / Q000150:complications* / Q000188:drug therapy; D014456:Ulcer / Q000209:etiology
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone