O Zehou1, J-J Raynaud2, C Le Roux-Villet1, M Alexandre1, G Airinei2, F Pascal1, M Heller3, N Lièvre3, L Laroche1, F Caux1, R Benamouzig2, C Prost-Squarcioni1,3,4. 1. Department of Dermatology and Referral Center for Auto-Immune Bullous Diseases, Avicenne Hospital, APHP, University Paris 13, 93009, Bobigny, France. 2. Department of Gastroenterology, Avicenne Hospital, APHP, University Paris 13, 93009, Bobigny, France. 3. Department of Histology and Referral Center for Auto-Immune Bullous Diseases, UFR Léonard de Vinci, University Paris 13, 93017, Bobigny, France. 4. Department of Pathology, Avicenne Hospital, APHP, University Paris 13, 93009, Bobigny, France.
Abstract
BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: To characterize systematically the endoscopic lesions of a series of patients with oesophageal symptoms seen at a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred patients with MMP with oesophageal involvement, systemic and endoscopic treatments, and follow-up are described. RESULTS: Of 477 consecutive patients with MMP consulting between 2002 and 2012, 26 (5·4%) had symptomatic oesophageal involvement. Dysphagia, observed in 23 (88%) patients, was the most frequent symptom. Oesophageal symptoms could be the first sign of MMP. Patients with oesophageal involvement had a mean of three other involved sites. At initial oesophageal endoscopy, 17 of 26 patients had active lesions (intact bullae, erosions and/or erythema), 15 had stricture(s) and 12 had other cicatricial lesions. Systemic therapy alone achieved oesophageal symptom relief for five patients. Dilatation was combined with systemic therapy for 12 patients and was successful in nine; one perforation occurred. CONCLUSIONS: Symptomatic oesophageal involvement affected 5·4% of patients with MMP. Dermatologists and gastroenterologists should be aware of these mucocutaneous diseases and their oesophageal involvement, as it could lead to earlier diagnosis and better care. Oesophageal dilatation could be a therapeutic option for symptomatic stricture not relieved by optimized systemic therapy alone.
BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: To characterize systematically the endoscopic lesions of a series of patients with oesophageal symptoms seen at a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred patients with MMP with oesophageal involvement, systemic and endoscopic treatments, and follow-up are described. RESULTS: Of 477 consecutive patients with MMP consulting between 2002 and 2012, 26 (5·4%) had symptomatic oesophageal involvement. Dysphagia, observed in 23 (88%) patients, was the most frequent symptom. Oesophageal symptoms could be the first sign of MMP. Patients with oesophageal involvement had a mean of three other involved sites. At initial oesophageal endoscopy, 17 of 26 patients had active lesions (intact bullae, erosions and/or erythema), 15 had stricture(s) and 12 had other cicatricial lesions. Systemic therapy alone achieved oesophageal symptom relief for five patients. Dilatation was combined with systemic therapy for 12 patients and was successful in nine; one perforation occurred. CONCLUSIONS: Symptomatic oesophageal involvement affected 5·4% of patients with MMP. Dermatologists and gastroenterologists should be aware of these mucocutaneous diseases and their oesophageal involvement, as it could lead to earlier diagnosis and better care. Oesophageal dilatation could be a therapeutic option for symptomatic stricture not relieved by optimized systemic therapy alone.
Authors: H Rashid; A Lamberts; L Borradori; S Alberti-Violetti; R J Barry; M Caproni; B Carey; M Carrozzo; F Caux; G Cianchini; A Corrà; G F H Diercks; F G Dikkers; G Di Zenzo; C Feliciani; G Geerling; G Genovese; M Hertl; P Joly; A V Marzano; J M Meijer; V Mercadante; D F Murrell; M Ormond; H H Pas; A Patsatsi; C Prost; S Rauz; B D van Rhijn; M Roth; E Schmidt; J Setterfield; G Zambruno; D Zillikens; B Horváth Journal: J Eur Acad Dermatol Venereol Date: 2021-07-10 Impact factor: 6.166