An algorithmic approach for the management of hand deformities in dystrophic epidermolysis bullosa

Abstract

Treatment of hand deformities in epidermolysis bullosa patients represents a challenging field in hand surgery practice, thus a systematic approach by a team is mandatory for a successful result. A simple and practical algorithm for the surgical treatment of hand deformities in EB was employed by the authors where the deformities of each digit in EB patients was categorized according to pseudosyndactyly and interphalangeal joint contracture severity for guidance during the surgical treatment. The current study retrospectively reviewed the medical records and photographic data of 13 EB patients followed in our department, for whom a systematic approach to the management and treatment was used. Mild cases were treated by surgical release and secondary healing with non-adhesive dressing while moderate cases were treated with autologous dermal grafts harvested with a special technique that were fixed on denuded areas on the proximal interphalageal joints after release. The remaining areas were treated similarly to the mild group. Additional K-wires were applied for two weeks in severe cases. A total of 21 procedures were performed on 13 EB patients with hand deformities according to the proposed treatment strategy. Functional recovery was satisfactory for each patient and the outcomes were dependent upon the severity of deformity. A multidisciplinary and conscious approach followed by an algorithmic surgical treatment protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients.

Keywords:

• Coccoon deformity
• dermal graft
• epidermolysis bullosa
• mitten deformity
• pseudosyndactyly

Disclosure statement

None of the authors of this paper have a conflict of interest or any disclosures. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.