RELEASE OF ACQUIRED SYNDACTYLIES IN THE KINDLER SYNDROME

Kindler syndrome is a rare, blistering skin disease characterized by acral bullae, poikiloderma, and diffuse cutaneous atrophy. Kindler syndrome has been established as a separate entity from epidermolysis bullosa; however, controversy still remains as to whether Kindler syndrome can be differentiated from Weary's hereditary acrokeratotic poikiloderma. Fusion of the digits secondary to blistering and scarring, ''pseudosyndactyly,'' has been reported in several patients with Kindler syndrome; however, surgical correction of the syndactylies in these patients has not been described. In this report, a patient with Kindler syndrome underwent surgical treatment of acquired syndactylies. Treatment included a tailored approach to preparation of the patient for surgery, surgical separation of fused tissues, selection of donor site for skin-graft harvest, postoperative dressings, splinting, and therapy. Results in our patient 2 years after correction demonstrate that syndactyly release in Kindler syndrome can be accomplished effectively, with improvement in both function and appearance.