Abstract

YESENIA AREVALO, Niní; BUJAN, María M; CERVINI, Andrea B  and  PIERINI, Adrián M. Bazex-Dupré-Christol syndrome: Case series. Arch. argent. pediatr. [online]. 2015, vol.113, n.5, pp.e256-e259. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2015.e256.

Bazex-Dupré-Christol syndrome is an X-linked dominantly inherited disorder characterized by congenital hypotrichosis, hypohidrosis, follicular atrophoderma, multiple milia and basal cell carcinomas. We present a girl and her family with this syndrome. Our patient, her 5 month old brother and her 17 year old brother had multiple milia and scalp and eyebrows hypotrichosis. Her 8 year old brother had multiple milia and follicular atrophoderma. Her mother had hypohidrosis and congenital scalp and eyebrows hypotrichosis, as well as a right paranasal lesion suggestive of basal cell carcinoma. We emphasize the importance of precise diagnosis and clinical follow up of these patients due to the possibility of developing basal cell carcinomas.

Keywords : Bazex-Dupré-Christol syndrome; Hypotrichosis; Hypohidrosis; Follicular atrophoderma and basal cell carcinoma.

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