Peculiar deformity of hands occurring in epidermolysis bullosa.

Abstract

THE DISEASE known as epidermolysis bullosa was first described by Tilbury Fox in 1879. 1 Since then many case reports have been published, and, although the disease is rare, individual cases are not now worthy of reporting unless they present some unusual feature. Efforts of classification into congenital types and acquired types, hereditary or nonhereditary, have been repeatedly made. Tulipan 2 divided the chief types into three groups: simplex, dystrophica (dominant) and dystrophica (recessive), according to the clinical features. Becker and Obermayer 3 recognized only two forms: epidermolysis bullosa simplex and epidermolysis bullosa dystrophica. Most recorded cases have presented the following common features: The condition appears at birth, or shortly afterward, and bullae appear in response to slight trauma and most frequently on the parts most exposed to trauma—the extremities, especially near joints. We herein report a rare case showing a peculiar deformity of the hands resulting from