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The occurrence of pyloric atresia in association with congenital epidermolysis bullosa (EB) has been reported in more than 80 patients. Almost all of these cases are in patients with junctional EB of a special subset characterized by abnormalities of the 64-integrin by mutation of either the 6 or the 4 chain, resulting in both cases in a defective link between the basal keratinocytes and the anchoring filaments of the lamina lucida.¹ We present an uncommon case of congenital pyloric stenosis in a young patient associated with a dominant dystrophic form of EB and a congenital localized absence of skin (Bart syndrome).

Report of a Case

An 8-month-old boy was referred for mucous and acral blistering since birth. His familial background was remarkable for early and regressive acral trauma–induced blisters, atrophic scars on the dorsal aspects of feet and hands, cutaneous brittleness, and residual, long-lasting onychodystrophy of most fingers in his father, his paternal . . . [Full Text of this Article]

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