Surgical management of hand deformities in hereditary dystrophic epidermolysis bullosa
نویسندگان
چکیده
منابع مشابه
The surgical management of dystrophic epidermolysis bullosa (excluding the hand).
Fifty patients with Dystrophic Epidermolysis Bullosa (DEB) underwent surgery including release of limb, oral, anal, eye and penile contractures and treatment of chronic skin ulceration or skin tumours. Correction of contractures involves extensive release of skin and underlying tissues, with split skin grafting of secondary defects. Specific regions are discussed. Recurrence is inevitable due t...
متن کاملPretibial dystrophic epidermolysis bullosa*
Epidermolysis bullosa is a group of mechano-bullous genetic disorders caused by mutations in the genes encoding structural proteins of the skin. Dystrophic epidermolysis bullosa is caused by mutations in the COL7A1 gene encoding collagen VII, the main constituent of anchoring fibrils. In this group, there are autosomal dominant and recessive inheritances. The pre-tibial form is characterized by...
متن کاملTreatment of severe hand deformities caused by epidermolysis bullosa.
Little research has been done regarding the treatment of severe hand deformities caused by epidermolysis bullosa. A 14-year-old boy was diagnosed with congenital epidermolysis bullosa. He was treated in our hospital several times, but the pathogenetic condition worsened. On examination, both hands were clenched fists and had scar formation. Skin fusion was observed between the 5 fingers. Nails ...
متن کاملSuprathel(®) -assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient.
Epidermolysis bullosa (EB) is a progressive familial disorder composed of dermal mucosal blisters, flexion contractures and pseudosyndactylies. Flexion contractures and pseudosyndactyly can be treated with surgery but usually require skin grafting. Because of poor wound healing, skin graft harvesting is a challenge in these patients. In order to prevent donor-site morbidities due to skin graft ...
متن کاملJunctional and Dystrophic Epidermolysis Bullosa
Epidermolysis bullosa (EB) is a congenital genodermatosis, which affects mainly skin and occasionally other organs [1]. Lifelong blistering and erosion of the skin and mucous membrane, caused by mechanical trauma, threaten EB patients [1]. The most common cause of death is metastasizing squamous cell carcinoma [2]. EB is subdivided into mainly three categories by the location of tissue separati...
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ژورنال
عنوان ژورنال: Military Medical and Pharmaceutical Journal of Serbia
سال: 2003
ISSN: 0042-8450,2406-0720
DOI: 10.2298/vsp0301011p