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2014, Number 2

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Cir Plast 2014; 24 (2)

Surgical treatment of pseudo syndactyly in patients with recessive dystrophic epidermolysis bullosa

Montemayor-Sánchez D, Salas-Alanís JC

Language: Spanish
References: 40
Page: 106-112
PDF size: 372.40 Kb.


ABSTRACT

Pseudosyndactyly of the hands and feet is an inevitable complication of patients with recessive dystrophic epidermolysis bullosa. This inherited disease is the result of mutations in the Type VII collagen (Col VII A1) resulting in blistering secondary to minor trauma. This condition of the hand is characterized by the formation of scars and hand deformities. If untreated, the hand may become encased in an epidermal cocoon. Currently, the recurrence of skin lesions is inevitable and surgical treatment focuses on maintaining a functional hand decreasing its progression to pseudosyndactyly. This article is a review of the pathophysiology and surgical management of patients with pseudo syndactyly in recessive dystrophic epidermolysis bullosa, aimed at improving the management of this condition.


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