Introduction and Objective: Recessive dystrophic epidermolysis bullosa (RDEB) is a
serious hereditary condition (mutations within the COL7A1 gene) characterized by
chronic inflammation, where the skin is prone to the development of chronic
wounds, scarring, and fibrosis that, in the final state, lead to progressive syndactyly,
thumb adduction contractures, and mitten hand deformities. This article highlights
the latest surgical techniques, rehabilitation methods, and additional therapies,
focusing on patients' autonomy, caregiver burden, and social engagement. Methods
used for the literature review in academic style: A thorough literature review was
performed to extract data from journals, guidelines, and regulations of regulatory
organizations for the period 1992-May 2026. The literature review concentrated on
such themes as RDEB, hand surgery, dressings, splints, occupational therapy,
physiotherapy, wound care, pain control, and wound healing innovations. Current
Status: Surgery produces improvements in function regarding grasping and
pinching functions but has a high recurrence rate due to skin fragility and scar
formation associated with wound healing. Outcomes rely on proper wound care,
minimizing trauma through splinting after surgery, hand therapy, education of
caregivers, pain management, and the functional use of the hand. Current wound
healing approaches make it possible for wounds to heal but do not reverse existing
contractures or pseudosyndactyly. Conclusion: Management of the mitten-glove
hand in RDEB must be multidirectional, involving surgical management,
rehabilitation, occupational retraining, and other adjunctive treatments in order to
reduce the burden of the wound and restore function.
Keywords: recessive dystrophic epidermolysis bullosa; mitten-glove hand;
pseudosyndactyly; hand surgery; rehabilitation; wound healing
