[Dystrophic epidermolysis bullosa: surgical treatment of advanced hand deformities].

Dystrophic epidermolysis bullosa (Hallopeau-Siemens, recessive dystrophic epidermolysis bullosa) is a rare inherited disorder of the skin and mucosa, characterized by blistering in response to the slightest mechanical trauma. Healing is associated with scarring and the formation of contractures and milia. Following repetitive trauma (friction), this process leads to severe hand deformities with digits contracted in flexion, the thumb contracted in adduction and pseudo-syndactyly.