Epidermolysis Bullosa (EB) is a very rare genetic connective tissue disorder resulting from a defect in the anchoring between the epidermis and dermis. The disease is manifested by extremely fragile skin that blisters and tears from minimal friction or trauma. The severity of the disease can range from mild to lethal. Internal organs and bodily systems can also be affected, creating multiple secondary complications.
Hand deformities occur in most patients with dystrophic EB and include adduction contractures of the first web space, pseudosyndactyly, and flexion contractures of all joints. This palmar view of a hand shows adduction of the thumb (top right) and fully flexed fingers (top center) encapsulated by the skin. Meticulous skin care and the use of well-fitted custom orthoses as part of a multidisciplinary team treatment approach are essential to maintaining hand function.
Information is available from the Dystrophic Epidermolysis Bullosa Research Association of America at http://www.debra.org/whatiseb#sthash.6zpsJMTA.dpuf.
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