Abstract
BackgroundEpidermolysis bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma. A major and common EB subtype, recessive dystrophic EB (RDEB), is characterized by altered wound healing, inflammatory dysbalance and fibrotic changes associated with reduced to absent collagen VII. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represent a challenging field in hand surgery practice.MethodsThe authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with an innovative “glove protocol” using Integra® dermal regeneration template. The endpoints analyzed were: healing times, hospital stay time, discomfort for the patient, free-recurrence interval, follow-up range and major complications.ResultsA total of 34 procedures were performed on 24 RDEB patients with hand deformities. Compared with the dressing with vaseline gauze, microsurgery followed by application of dermal regeneration template gloves allowed a significant reduction of hospital stay, healing time, and dressing pain as well as an increased recurrence-free interval.ConclusionsThe microsurgical approach followed by our new protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients.
Highlights
Epidermolysis bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma
Recessive dystrophic EB (RDEB) is the more frequent DEB subtype with a prevalence of about 2 in 1,000,000 population [2, 5] and it is related to the loss of collagen VII expression that causes generalized skin blisters at birth, involvement of mucous tissue, chronic anemia, poor nutrition, recurrent infection, severe renal complication and squamous cell carcinomas [6]
For example, the repeated minimal trauma, the abnormal inflammatory dysbalance and the fibrotic changes associated with lack of collagen VII lead to pseudosyndactyly with partial and / or complete loss of the interdigital spaces, flexion contractures of the joints and adduction contracture of the thumb [8, 9]
Summary
Epidermolysis bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma. Recessive dystrophic EB (RDEB) is the more frequent DEB subtype with a prevalence of about 2 in 1,000,000 population [2, 5] and it is related to the loss of collagen VII expression that causes generalized skin blisters at birth, involvement of mucous tissue (gastrointestinal tract), chronic anemia, poor nutrition, recurrent infection, severe renal complication and squamous cell carcinomas [6]. This disease has a devastating impact on the quality of life of patients and requires multi-disciplinary team support [7]. The severe form of contractures is known as “mitten hand” or “cocoon hand” deformity
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