A new protocol for the treatment of hand deformities in recessive dystrophic epidermolysis bullosa (13 cases)

Campiglio, Gianluca; Pajardi, G.; Rafanelli, G.

doi:10.1016/s0753-9053(97)80025-7

Cited by 19 publications

(24 citation statements)

References 16 publications

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“…So, an early surgical approach is of paramount importance. In our experience, but also in literature, pseudosindatiles in patients with this disease are characterized by high rates of recurrence [6][7] . The scar tissue of the hands can alter the anatomical structures, including the vascular-nerve pedicels.…”

Section: Discussionsupporting

confidence: 65%

New protocol treatment of pseudo-syndactyly in the Epidermolysis Bullosa hand using the dermal regeneration template glove

Lembo¹,

Parisi²,

Cecchino³

et al. 2020

Preprint

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BACKGROUNDː The Epidermolysis Bullosa (EB) is a rare congenital disease associated with blistering skin caused by minimal trauma. 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 represents a challenging field in hand surgery practice.METHODSː The Authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with our innovative “glove protocol” using a 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.RESULTSː A total of 34 procedures were performed on 24 EB patients with hand deformities according to the proposed treatment strategy. We observed reduction of: hospital stay time, complete healing times, discomfort after dressings and average time to free recurrence.CONCLUSIONSː The 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.

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Section: Discussionsupporting

confidence: 65%

New protocol treatment of pseudo-syndactyly in the Epidermolysis Bullosa hand using the dermal regeneration template glove

Lembo¹,

Parisi²,

Cecchino³

et al. 2020

Preprint

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“…Epidermolysis bullosa dystrophica is a rare hereditary disease, with an estimated incidence of approximately 1 in 300,000 live births, and the disease affects mostly the stratified squamous epithelium 4,7,8 . The dystrophic scarring in RDEB is the most prominent and often leads to the development of hand deformities, and has the greatest degree of blistering and ulceration 1,2,5,9 .…”

Section: Discussionmentioning

confidence: 99%

“…The pathway for SCC development in these cases may be attributed to the frequent malignant degeneration of the stratified squamous epithelium in the adulthood. Also, repeated injury and healing of damaged skin, scarring, and chronic wounds such as nonhealing ulcers may play a role in the development 4,7,10,11 . According to some authors, SCC tends to grow and spread faster in people with RDEB than in those without the disease 3,5 , and may primarily occurs in the hand, often develop over the dorsum of the joints and tends to be extremely aggressive and surgical excision is the only effective therapy 2,3,6 .…”

Section: Discussionmentioning

confidence: 99%

“…The DEB may be transmitted as an autosomal dominant or recessive (RDEB) subtype 2 . The RDRB or scaring type is the most severe, which produce blistering, ulcerations, fibrinous adhesions, with slow or non-healing chronic lesions, also creates severe hand deformities 1,2,[4][5][6] , and it is not uncommon for SCC to develop at the site of chronic hand lesions in patients with severe RDEB who survive to childhood 2,5,6 .…”

Section: Introductionmentioning

confidence: 99%

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A Report of Two Cases of Squamous Cell Carcinoma of the Hand in Patients With Epidermolysis Bullosa Dystrophica

Muradian¹

2016

Basrah Journal of Surgery

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This is a report of two patients (brothers) with unusual generalized skin disorder, a recessive dystrophic epidermolysis bullosa (RDEB) that appeared at childhood, who developed a progressive squamous cell carcinoma (SCC) in their right hands. Later both patients underwent wide local tumor excision, and during the follow-up after surgery, axillary lymph node metastasis observed in the first case, and local recurrence in the second. Therefore early recognition of EBD is important to assist in the diagnosis and treatment of malignant lesions at an early stage in these patients to prevent the subsequent complications.

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“…This treatment is less traumatic and less stressful for the patient. [7] There is no consensus concerning the use of skin substitutes such as Biobrane™[9] or keratinocyte cultures[13] in the literature. Moreover, their high cost limits their use.…”

Section: Discussionmentioning

confidence: 99%

Inherited epidermolysis bullosa: Case report of finger localization

Sankale

Coulibaly

Ndiaye

et al. 2012

Indian J Plast Surg

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Inherited epidermolysis bullosa is a rare condition that often present at birth with skin blisters and erosions. They are associated with defective cohesion of the dermis and epidermis. There are 3 principal types: Simple, junctional and dystrophic. The severity of the condition is quite variable. The most severe forms are incompatible with life. The most common types in our country are the severe ones such as the Hallopeau -Siemens subtype. Hands and mucosal areas can develop synechia. We report here a case of dystrophic epidermolysis bullosa in a 27-year-old woman whose finger lesion was managed surgically. This treatment consisted of complete removal of constrictions and adhesions, accompanied by use of a Hueston flap and skin graft to repair the tissue deficit. The patient's clinical course required several repeat operations. This surgery allowed the possible total loss of hand function to be delayed but the inevitable progression of the illness made the treatment somewhat disappointing. Psychosocial implications are very significant in our setting.

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A new protocol for the treatment of hand deformities in recessive dystrophic epidermolysis bullosa (13 cases)

Cited by 19 publications

References 16 publications

New protocol treatment of pseudo-syndactyly in the Epidermolysis Bullosa hand using the dermal regeneration template glove

New protocol treatment of pseudo-syndactyly in the Epidermolysis Bullosa hand using the dermal regeneration template glove

A Report of Two Cases of Squamous Cell Carcinoma of the Hand in Patients With Epidermolysis Bullosa Dystrophica

Inherited epidermolysis bullosa: Case report of finger localization

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