Abstract
REPORT OF A CASE Since a few months after birth, this 43-year-old man has reportedly suffered from sporadic blistering that occurs both spontaneously and in response to trauma. The blistering generally heals without scar formation. The patient has a younger brother with the same disease; their parents are first cousins. When first seen in our department in March 1986, the proband was noted to have multiple blisters, erosions, and crusts located primarily on the lower legs. Numerous atrophic scars, diffusely distributed over the entire body, were also observed, although milia were absent. The nails of both the fingers and the toes were either dystrophic or missing. The teeth were normal; however, many of the patient's teeth were extracted because of early caries. The scalp and hair were found to be normal, and no lesions were found on the oral mucosa. Histologic examination of a fresh lesion showed a subepidermal blister
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