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SAS Journal of Surgery | Volume-10 | Issue-04
Surgical Management of Psudosyndactyly in Recessive Dystrophic Epidermolysis Bullosa: About Three Case Reports
Badr Rouijel, Abir Manni, Nawfal Fejjal
Published: April 30, 2024 | 51 42
DOI: 10.36347/sasjs.2024.v10i04.025
Pages: 506-511
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Abstract
Recessive dystrophic epidermolysis bullosa (RDEB) is defined as a congenital disorder resulting from mutations in the COL7A1 gene, often resulting in hand contractures and pseudosyndactyly. While multiple treatments exist to improve hand malformations, there are currently still no radical cures for this disease because of its high recurrence rate. This paper reports our experience of management of hand deformities in three cases of Recessive dystrophic epidermolysis bullosa patients with surgical management and postoperative skin dressings. Hand function was significantly improved after the complete release of pseudosyndactyly and the achievement of satisfying digital web spaces. Patients were followed up for one year and showed good results after functional hand training activities.