Evaluation of Non-coverage Cost Effective Technique for Management of Epidermolysis Bullosa Mitten Hands in Developing Countries

Abstract

Background: Epidermolysis bullosa (EB) is a group of rare genetic disorders known by susceptibility of the skin epidermis and mucosa that separate from underlying tissues and cause increase in skin fragility, blistering, and scarring in response to minor traumatic injuries. Many methods were described in the literatures for soft tissue coverage after the release of mitten hands which include cultured keratinocytes; using epithelium raised from cocoon, to split skin grafts or full-thickness skin grafts; and cellular allograft dermal matrix. In this study, we described a simple and efficient non-coverage technique, as it eliminates the donor site complications when using STSG or FTSG, and also minimizes cost management by avoid using cellular allograft dermal matrix, specially in developing countries, where cost effectiveness due to financial issues should be considered.Aim of this study: is to describe and evaluate the non-coverage technique based on the Birmingham Epidermolysis Bullosa Severity Score Sheet (BEBSS) in patients suffering from epidermolysis bullosa mitten hands.Patients and Methods: We operated upon nine patients suffering from epidermolysis bullosa mitten hands by using the non-coverage technique to obtain full release of all fingers and web spaces. Evaluation and scoring of preoperative and postoperative hand scars were based on the ‘Birmingham Epidermolysis Bullosa Severity Score Sheet.Results; showed major improvement in the mean of post-operative BEBSS with lower rate of recurrence and postoperative complications. Hand function was remarkably improved within 2 months which was demonstrated subjectively by the capability of grasping and pinching objects such as cups, spoons and pens using unilateral hand.All patients and care givers were satisfied with the improvement in function and the ability to perform more tasks than preoperatively.Conclusion: Managing soft tissue defects after release of EB mitten hands can be left with no coverage and with appropriate dressing for self-reepithelization, can be ideal method especially in developing countries where allografts are relatively expensive and not available.