Involvement of the Hand in Epidermolysis Bullosa

Horner, Robert L.; Wiedel, Jerome D.; Bralliar, F

doi:10.2106/00004623-197153070-00010

Cited by 21 publications

(7 citation statements)

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“…Initially presenting as partial proximal web formation within the interdigital spaces of the hands and feet, progression may eventually lead to total encasement of the digits within a cocoon (''mitten'')-like keratinized sac, resulting in marked impairment of hand function and difficulties in weight bearing, standing, and walking. Earliest reports were based on case reports, 1,2 small case series, [3][4][5] or limited cohorts [6][7][8][9][10] of EB patients selected for and referred to specialized plastic and orthopedic surgical centers for reconstruction. In one of the more recently published series, involving 19 children between 2 and 14 years of age who were referred for hand surgery, 9 all had some degree of pseudosyndactyly and contracture; 12 of 19 had advanced cocoon formation.…”

mentioning

confidence: 99%

“…Most of the earlier studies described degloving of the hands and immobilization of the digits, followed by grafting with either split-thickness grafts 4,7,13,14 or full-thickness skin. 6 Other variations on this technique have been subsequently described, to include the use of artificially prepared autologous skin graft sheets 5 or no grafts.…”

mentioning

confidence: 99%

“…Second, recurrence is the norm, seen in about half of all treated patients. 7,9,10,16 If optimal function is to be maintained, additional procedures are required about every 2 years. 8,11,14 As a correlate, recurrences may be more frequent in the dominant than nondominant hand.…”

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confidence: 99%

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Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

241

178

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confidence: 99%

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confidence: 99%

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Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

241

178

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“…Surgery and release of pseudosyndactyly allows independent finger motion and helps improve the aesthetic appearance of hands [ 7 , 24 ]. A case report, reported no post-operative improvement, resulting in not recommending release of pseudosyndactyly [ 25 ].…”

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

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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.

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“…In some patients, newer techniques of reconstructive hand surgery may lead to significantly increased function, though unfortunately such procedures may have to be performed repeatedly (Horner et al, 1971). The mainstay of management of most patients with dystrophic EB remains symptomatic and supportive therapy.…”

Section: Discussionmentioning

confidence: 99%

The musculoskeletal manifestations of epidermolysis bullosa: an analysis of 19 cases with a review of the literature

Wong

Pemberton²

1992

The British Journal of Radiology

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Epidermolysis bullosa encompasses a group of rare disorders characterized by marked skin fragility and blister formation. In patients with dystrophic epidermolysis bullosa, skeletal and soft-tissue abnormalities are an important feature. An analysis of the musculoskeletal manifestations in 19 patients is presented. In the hands and feet, features included generalized osteoporosis, wedge-shaped thinning and hooking of distal phalanges, overconstricted bones, acro-osteolysis, flexion contractures, metatarsal and metacarpal subluxation, distal trophic changes, webbing of digits, encasement of the whole extremity in a pouch of skin, soft-tissue calcification and retarded skeletal maturity. Previously undescribed findings in the hands and feet are bony ankylosis of the proximal interphalangeal joints, resorption of the metatarsal and metacarpal heads, shortened metatarsal bones, carpal and tarsal fusion and destruction, and cystic changes of the distal radius and ulna. In the remainder of the skeleton, hip dysplasia with premature osteoarthritis, knee joint bony ankylosis and thoracic and thoraco-lumbar scoliosis are other undescribed findings.

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Involvement of the Hand in Epidermolysis Bullosa

Cited by 21 publications

References 0 publications

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

The musculoskeletal manifestations of epidermolysis bullosa: an analysis of 19 cases with a review of the literature

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