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Case Rep Dermatol. 2017 Aug 3;9(2):112-118. doi: 10.1159/000478889. eCollection 2017 May-Aug.

A Case of Aplasia Cutis Congenita Type VI: Bart Syndrome.

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1
Dermatology Department, King Fahad Armed Force Hospital, Jeddah, Saudi Arabia.

Abstract

Aplasia cutis congenita type VI, also known as Bart syndrome, is a rare genetic mechanobullous disorder characterized by congenital localized absence of skin, mucocutaneous blistering lesions, and nail abnormalities. We present the case of a 4-h-old male newborn who presented with complete absence of skin over the anteromedial aspect of both lower legs associated with nail dystrophy since birth. After a few days, he developed blisters that were consistent with epidermolysis bullosa in histopathological examination. There was no systemic involvement such as pyloric atresia, ureteral stenosis, renal abnormalities, or arthrogryposis. All laboratory work and imaging studies were normal. A diagnosis of Bart syndrome was made based on previous presentation. We managed the patient with conservative methods. Complete epithelialization occurred after several weeks.

KEYWORDS:

Aplasia cutis congenita type VI; Bart syndrome; Conservative treatment; Epidermolysis bullosa

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