Purpose: To review the oral prosthetic rehabilitation of patients with epidermolysis bullosa hereditaria (EBH) and study the level of evidence and quality of the presented available articles and establish clinical recommendations for the prosthetic management of these patients. Materials and Methods: An electronic search was done in July 2022 in five databases following PICOTS elements. The quality of the reports was established using the modified Pierson, Bradford Hills, and Ottawa Newcastle scale. Results: Data extracted from 19 case reports for protocolized analysis corresponded to 64 patients and 80 dental prostheses, with almost 9 out of 10 patients being completely edentulous. The distribution of EBH types was 84% dystrophic, 10.5% junctional, and 5.5% simplex. The difficulties encountered by the authors synthesized in this review characteristically reflected those most likely encountered in dental practice. Most rehabilitations were implant-supported prostheses (85%), followed by removable dentures (10%) and dental-supported rehabilitations (5%). Fixed full-arch implant-supported prostheses represented 76.4% of implant-supported prostheses, and this prosthetic solution reflected the highest scientific quality. Conclusions: For an individualized approach to treatment, we recommend that in cases of total edentulism, fixed full-arch implant-supported prostheses are the most appropriate because they allow the best computer-aided planning, design, manufacture, and fitting of the prosthesis in such a complex clinical context.
