The hand in recessive dystrophic epidermolysis bullosa

Recessive dystrophic epidermolysis bullosa is a difficult challenge for surgeons. The aim of the surgical procedure is to release the adduction contracture of the thumb, and to separate the pseudosyndactyly to release the contracture of the fingers to improve hand function, particularly pinch and grip. Surgery is performed in three steps. Without postoperative physiotherapy, recurrence of retractions is unavoidable. Prolonged physiotherapy with the use of dynamic splints is useful.