Successful forearm prosthesis fitting in a patient with epidermolysis bullosa dystrophica: Case report. (June 2018)

• Record Type: Journal Article
• Authors: Verduyn, Carl; Morren, Marie-Anne; Stas, Marguerite; Sinnaeve, Friedl; Segers, Katarina; Kiekens, Carlotte
• Is Part Of: Prosthetics and orthotics international. Volume 42:Number 3(2018)
• Page Start: 254
• Page End: 257
• Publication Date: 2018-06
• Subjects: Epidermolysis bullosa -- squamous cell carcinoma -- amputation -- prosthesis -- rehabilitation -- shared decision making; Orthopedic apparatus -- Periodicals; Prosthesis -- Periodicals; Implants, Artificial -- Periodicals; 617.905
• DOI: 10.1177/0309364617718410
• Languages: English
• ISSNs: 0309-3646

Abstract

Background: Epidermolysis bullosa dystrophica is a rare dermatological disease characterized by extreme skin fragility and elevated risk of developing a squamous cell carcinoma. In some cases, amputation of a limb is necessary. Case description and methods: A 37-year-old man with recessive, severe generalized epidermolysis bullosa dystrophica developed a squamous cell carcinoma on the right forearm requiring a below-elbow amputation. Preoperative advice concerning indication and level of amputation was given. Due to potential skin problems, a conventional prosthesis was not feasible. Findings and outcomes: A custom-designed adaptive prosthesis with an upper arm cuff was trialed and was well tolerated. Multiple working tools, attached with a rotation and inclination system, allowed independence and return to work. Conclusion: Despite multiple potential skin problems of the stump, the patient was successfully fitted with a custom-designed adaptive prosthesis. Preparation for this fitting was done by a comprehensive multidisciplinary patient-centered approach. Clinical relevance: Despite severe skin fragility, a patient with epidermolysis bullosa dystrophica was successfully fitted with a custom-designed adaptive upper limb prosthesis allowing good functional outcome. This required a multidisciplinary and patient-centered approach.