Overview of Complications Associated with Epidermolysis Bullosa: A Multicenter Retrospective Clinical Analysis of 152 Cases
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In brief
In brief
Multicenter retrospective analysis of 152 Saudi children with epidermolysis bullosa reveals subtype-specific surgical complications, with dystrophic and junctional forms showing higher rates of esophageal strictures, pseudosyndactyly, and skin malignancies. Study emphasizes the critical role of surgeons in EB management and the importance of subtype recognition for predicting outcomes and treatment response.
Written by the GCMD Library team from the article.
Background/Purpose
Epidermolysis bullosa (EB) is a rare disease of skin and mucosa which may causes surgical complications. We review these in a large patient cohort from Saudi Arabia.
Methods
A retrospective study was conducted at 21 centers between 2003 and 2020. Demographic data and information on EB type [Simplex (EBA), Dystrophic (DEB) and Junctional (JEB)]. The dataset included clinical features, operations, surgical complications, and treatment.
Results
There were 152 (63 male) children with EB [EBS n = 93 (61.2%); DEB n=30 (19.7%); JEB n=25 (16.4%), and Kindler syndrome n = 4, (2.6%)]. Children with JEB and DEB tended to have a higher frequency of skin and musculoskeletal system complications (skin cancer, pseudosyndactyly and recurrent skin infection). Esophageal strictures were mostly seen in DEB (n = 19, 63%) and to a lesser extent in EBS (n =20, 21%) and JEB (n = 4, 16%). Pyloric atresia was uncommon (n=4) and limited to those with JEB. Percutaneous gastrostomy for feeding support was used in all types. Ankyloglossia was common but often recurred (76%) after division. Circumcision was usually safe and complication-free in male children except in those with severe JEB. Phimosis was reported in 10% of uncircumcised patients.
Conclusions
Our series showed that surgeons play a key role in the management of some complications associated with EB. It is also important to be aware of the particular sub-type as this can predict the natural history and likely response to treatment.