Vojnosanit Pregl
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In the period 1996-2001 in the Clinic for Plastic Surgery and Burns of the Military Medical Academy, 18 patients. 12 male and 6 female, with hereditary dystrophic epidermolysis bullosa (HDEB) and hand deformities were surgically treated, to achieve the complete separation of fingers, correction of the thumb adduction contracture and flexion or extension contracture of finger joints. The period of wound healing on flat surfaces after surgery, and the period between two operations was estimated. The most common deformity was the flexion contractures of metacarpophalangeal (MP) joints (45%) and one or both interphalangeal (IP) joints (types A1, A2). ⋯ EBDC represents great medical and social problem that requires multidisciplinary approach of physicians of various specialties (surgeons, dermatologists, pediatrists, geneticists, nutritionists, physiatrists, ophthalmologists, dentists, ENT, as well as specially trained persons and families). The efficient specific systemic therapy aiming to increase the skin resistance to mechanical trauma does not exist yet, and should be developed in the field of gene therapy. The surgical correction of hand deformities, acrylate glove use in the longer post operative period combined with physiotherapy, the active use of hands, the protection of injuries and skin care are the measures which prolong the period between the recurrence of contractures.