| Title: |
Management of oesophageal strictures in inherited epidermolysis bullosa: a clinical practice guideline |
| Authors: |
El Hachem, May; Caldaro, Tamara; Lara-Corrales, Irene; Pope, Elena; Jones, Rosie; Bageta, Maria L; Heaton, Jennifer; Martinez, Anna E; Ferreira da Rocha, Anna Carolina; Bodemer, Christine; Russo, Claudia; Tagkalidis, Peter; Brains, Lisa M; McGuirk, Simon P; Diamantopoulos, Athanasios; Feinstein, James A; Zambruno, Giovanna; Mayre-Chilton, Kattya; De Angelis, Paola |
| Contributors: |
Italian Ministry of Health |
| Source: |
British Journal of Dermatology ; volume 193, issue 3, page 394-404 ; ISSN 0007-0963 1365-2133 |
| Publisher Information: |
Oxford University Press (OUP) |
| Publication Year: |
2025 |
| Description: |
Inherited epidermolysis bullosa (EB) is a group of rare and complex genetic disorders characterized by fragility of the skin and mucous membranes. Specifically, the gastrointestinal tract is commonly involved with a range of complications, one of the most disabling being oesophageal strictures (OS). OS manifest with progressive dysphagia, which in turn contributes to malnutrition, chronic anaemia and growth delay, with high impact on the quality of life of patients and their families. DEBRA International has supported the development of clinical practice guidelines (CPGs) for different aspects of EB care. The present CPG aims to provide healthcare professionals and affected individuals and their caregivers with recommendations on diagnostic procedures, preventive measures and treatment of OS. An international multidisciplinary panel comprising clinical experts and patient and public involvement (PPI) representatives developed the CPG, following an international PPI survey and literature review. The GRADE methodology was adopted to define PICO (population, intervention, comparison, outcome) questions, implement a literature appraisal process and prepare recommendations. Three recommendations are focused on OS diagnosis, and seven on preventive nonpharmacological (diet, oral care and therapeutic education) and pharmacological measures (topical corticosteroids) and treatment procedures. Treatment focuses particularly on oesophageal dilatation and how to delay and manage disease relapses. It is expected that this CPG will contribute to improving the quality and equity of care for individuals affected with EB, and will hopefully foster clinical research to increase evidence, in particular on noninvasive OS treatment to prevent complications and delay relapses. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1093/bjd/ljaf191 |
| DOI: |
10.1093/bjd/ljaf191/63253187/ljaf191.pdf |
| Availability: |
https://doi.org/10.1093/bjd/ljaf191; https://academic.oup.com/bjd/advance-article-pdf/doi/10.1093/bjd/ljaf191/63253187/ljaf191.pdf; https://academic.oup.com/bjd/article-pdf/193/3/394/63253187/ljaf191.pdf |
| Rights: |
https://creativecommons.org/licenses/by-nc/4.0/ |
| Accession Number: |
edsbas.310CCB36 |
| Database: |
BASE |