Hand surgery in recessive dystrophic epidermolysis bullosa: Our experience with dermal substitutes

Abboud, Loubna; Leclerc‐Mercier, Stéphanie; Bodemer, Christine; Guéro, S.

doi:10.1016/j.bjps.2021.05.056

Cited by 8 publications

(5 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The use of genetically corrected autologous tissue engineered grafts looks even more promising. In the future, this may be a possible key to overcoming the tendency for recurrence of deformity [ 52 ].…”

Section: Resultsmentioning

confidence: 99%

“…A recent study described the use of full thickness skin graft at the level of the first commissure and palm of the hand, while dermal substitutes (Integra® or Matriderm®) were used to cover the remaining commissures, digits, and the rest of the hand, followed by a split thickness skin graft. Maintenance of function was greater than 3 years in 57% of cases, and greater than 5 years in 33% of cases [ 52 ].…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Box

Bernardis

Pleshkov

et al. 2022

Orphanet J Rare Dis

View full text Add to dashboard Cite

What is already known about this topic? Epidermolysis bullosa (EB) causes blistering and scarring of the hands resulting in contractures fused web spaces and altered function. Surgery is needed to release contractures and web spaces and hand therapy is essential to maintain results, approaches for both differ. What does this study add? These guidelines aim to provide information on the surgical and conservative therapeutic hand management of children and adults diagnosed with EB. They are based on available evidence and expert consensus to assist hand surgeons and therapists in decision making, planning and treatment. They highlight the importance of a holistic multidisciplinary team (MDT) approach, where patient priorities are paramount.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Box

Bernardis

Pleshkov

et al. 2022

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…DEB is associated with a genetic mutation of COL7A1 gene that encodes collagen type VII. (2) In turn, the sublamina densa layer of the basement membrane separates. (7) The estimated prevalence of DEB was reported to be 2-6 affected individuals per a million births and 1:1 male to female ratio.…”

Section: Discussionmentioning

confidence: 99%

“Management of Femur Shaft Fracture with Nancy Nail in the Setting of Dystrophic Epidermolysis Bullosa”: A case report

Tarabishi

Almonaie

Mohamed

et al. 2021

Preprint

View full text Add to dashboard Cite

BackgroundThe management of bone fractures must achieve both the reduction and stability providence. However, dermatological conditions such as dystrophic epidermolysis bullosa (DEB) for instance can lead to catastrophic events when operating on the patient’s bone fracture. This can lead to wound infections and possible failure of bone healing leading to fracture nonunion. This dermatological disorder leads to heterogenous bullous dermatoses including cutaneous fragility leading to cutaneous bullous formation after exposure to any type of trauma. DEB is a rare inherited form of the disease characterized by the formation of cutaneous bullae. DEB is associated with a genetic mutation of COL7A1 gene that encodes collagen type VII. Due to the rarity and uniqueness of the disease, special modifications due to the challenges faced during the patient care approach were accomplished to prevent any possible harm to the patient. In this study, we propose a case report that is followed by the anesthetic and surgical challenges faced and how they were modified upon.Case PresentationA 20-year-old female presented to the emergency department with a spiral mid-diaphyseal fracture of the femur after an incidence of falling from bed. The patient was previously diagnosed with dystrophic epidermolysis bullosa which made this case unique and complex. As the patient was examined by the team, her skin was covered with old blisters and wounds that have developed with the consequence of the disease. Due to the sophisticated dermatological condition and the unique presentation, the established multidisciplinary team took a decision to treat the patient with flexible intramedullary nailing in an open versus closed reduction technique, and modifications of the treatment approach were done based on the challenges in this case. The goal was to provide the management while minimizing the risk of infections and complications that would have arisen. The proposed case will set a baseline for the management of similar cases.ConclusionsWe suggest that in order to manage femur shaft fracture in the setting of dystrophic epidermolysis bullosa, modifying the management to avoid the least possible skin harm at any expense while managing the bone fracture is the golden approach.

show abstract

“…[8][9][10] Skin substitutes-acellular dermal matrices in particular-are often used as postsurgical healing strategies to quicken the healing process and reduce scarring tissue. [11][12][13] These tumors impact patients' life in terms of both overall and disease-free survival: in fact, cSCCs tend to relapse easily and reduce patient life expectancy. 14,15 The most recent guidelines for diagnosis and treatment of cSCCs in EB patients are based on expert consensus, but more data need to be collected in this setting.…”

Section: Introductionmentioning

confidence: 99%

“…Surgical excision is the standard of care but can be complicated by both the difficulties in identifying the tumor margins and the impairment in the wound‐healing process 8–10 . Skin substitutes—acellular dermal matrices in particular—are often used as postsurgical healing strategies to quicken the healing process and reduce scarring tissue 11–13 . These tumors impact patients’ life in terms of both overall and disease‐free survival: in fact, cSCCs tend to relapse easily and reduce patient life expectancy 14,15 …”

Section: Introductionmentioning

confidence: 99%

Surgical management and oncological follow‐up of cutaneous squamous cell carcinomas arising in epidermolysis bullosa patients

et al. 2022

View full text Add to dashboard Cite

Background Hereditary epidermolysis bullosa (EB) is a rare genodermatosis characterized by skin fragility and blistering of the skin and mucous membranes in reaction to minimal traumas. The development of cutaneous squamous cell carcinomas (cSCCs) is one of the most common medical complications in junctional and dystrophic forms of the disease.Complete surgical excision of cutaneous tumors represents the gold standard of treatment.However, not only recognition of cSCCs can be challenging in the affected skin but also wound closure after surgical excision poses a great therapeutic challenge in EB patients.The aim of our study was to analyze the postoperative outcomes of such patients in order to have a better knowledge of the main critical issues in their surgical management and oncological follow-up.Methods We retrospectively identified a cohort of five EB patients treated at Modena University Hospital. Collected data included patient age and sex, date of cSCC diagnosis, relapses/recurrences, site of the neoplasm, number of surgical interventions, use of dermal substitutes, and postoperative infections.Results A total of 26 cSCCs were detected in our cohort. Forty-one surgical interventions were necessary to achieve excision of cSCCs with clear margins, varying from 1 to 4 surgical sessions per cSCC. Dermal substitutes were used in most cases but carried a higher infectious risk.Conclusions EB patients tend to develop numerous cSCCs that often relapse even after complete excision with clear margins. These results stress the importance of early cSCC diagnosis and strict postsurgical follow-up. Materials and methodsA monocentric, retrospective analysis was performed on EB patients treated at the Dermatological Clinic of the Modena University Hospital. We included patients affected by EB with histologically diagnosed cSCCs and treated with surgical excision at our clinic, from inception to the present.We collected the following clinical data for each subject: age and sex, date of cSCC diagnoses, number of cSCCs, relapses, site of the neoplasm, number of surgical interventions needed

show abstract

Hand surgery in recessive dystrophic epidermolysis bullosa: Our experience with dermal substitutes

Cited by 8 publications

References 17 publications

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

“Management of Femur Shaft Fracture with Nancy Nail in the Setting of Dystrophic Epidermolysis Bullosa”: A case report

Surgical management and oncological follow‐up of cutaneous squamous cell carcinomas arising in epidermolysis bullosa patients

Contact Info

Product

Resources

About