Oesophageal Pemphigus vulgaris

Abstract

Symptomatic involvement of the oesophageal mucosa by pemphigus vulgaris is rare. We describe 1 patient who was treated with oral steroids during a blistering phase, when epigastric pain developed. Endoscopy revealed multiple ulcerations all over the oesophagus, but gastroduodenal mucosa was normal. The symptoms disappeared following cimetidine for gastro-oesophageal reflux and increase of steroid dosage. When painful symptoms appear from the upper digestive tract during corticosteroid treatment of pemphigus, the possibility of acantholytic involvement of oesophageal mucosa must be kept in mind. Its implication for the dosage of steroids is opposite that in steroid-induced peptic ulcers. Carefully performed upper gastrointestinal tract endoscopy is helpful in these patients.

Development of oesophageal involvement in a subject with pemphigus vulgaris: a case report and review of the literature

“…2,25,26 Otherwise, those same symptoms may be signs of a steroid-induced gastroduodenal condition that requires a different treatment regimen than that used for oesophageal pemphigus, which would thus result in the reduction of daily steroids with subsequent recrudescence of skin and mucosal lesions in cases of oesophageal pemphigus. 3 Delayed diagnosis or misinterpretation can be avoided by performing oesophagogastroduodenoscopy in selected cases; in particular in cases presenting a history of pemphigus vulgaris and who report oesophageal symptoms that do not respond to steroids and antacid /antisecretive adjustment. 2,22,27,28 The EGDS should be performed by a skilled endoscopist to avoid any examination-related exacerbation of the oesophageal lesions due to scope-induced positive Nikolsky sign.…”

“…1 Oesophageal involvement is rarely reported, perhaps because it is undetected or misdiagnosed as steroid-induced oesophagitis and peptic ulcers and subsequently treated with an inappropriate reduction of the daily steroid dose. 2,3 We report herein the case of a man in whom pemphigus vulgaris spread to the oesophagus after being confined for 5 years to the rhino-oro-pharyngeal mucosa and seborrhoeic areas of the skin.…”

“…The pharyngolaryngeal, genital, anorectal and conjunctival mucosae are less commonly involved 1 . Oesophageal involvement is rarely reported, perhaps because it is undetected or misdiagnosed as steroid‐induced oesophagitis and peptic ulcers and subsequently treated with an inappropriate reduction of the daily steroid dose 2,3 …”

Relapse of Pemphigus vulgaris Presenting with Haematemesis

“…There were no further episodes of haematemesis and the patient was discharged 48 h later. A repeat endoscopy 1 month later showed complete healing of the ulcer.Oesophageal involvement in pemphigus, though rare, has been reported in several publications [2][3][4][5][6][7][8]. In view of the fact that desmoglein proteins form the components of the intercellular substance of all squamous epithelia, the involvement of oesophageal mucosa can be easily explained [1].…”

“…Such patients actually need continuation or even enhancement of steroid dosage. The diagnosis is easily confirmed by endoscopy and hence it has been recommended that all such patients who present with upper gastro-intestinal symptoms should undergo the procedure [8]. Endoscopic biopsy is also helpful in distinguishing oesophageal pemphigus from candidal and herpetic oesophagitis, conditions which may occur in patients receiving long-tem steroid and immunosuppressive therapy.…”