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J Dtsch Dermatol Ges. 2004 Jul;2(7):602-4.

[Miliaria and follicular atrophodermia as an early sign of Bazex-Dupré-Christol syndrome].

[Article in German]

Author information

1
Klinik und Poliklinik für Hautkrankheiten des Universitätsklinikums Münster. beljan@uni-muenster.de

Abstract

The Bazex-Dupré-Christol syndrome (BDC syndrome) is a rare X-chromosomal dominantly inherited genodermatosis. Major symptoms are (1) follicular atrophoderma, (2) congenital hypotrichosis, and (3) multiple basal cell carcinomas, the latter appearing in the second or third decade. More variably associated findings are milia, hypohidrosis, disorders of cornification, hyperflexibility of joints, and skeletal abnormalities. A 2.5-year-old Turkish girl presented with early signs of BDC syndrome in the form of milia, follicular atrophoderma on the face and hands, and hypotrichosis. Accurate diagnosis of this genodermatosis is necessary to prevent the development of basal cell carcinomas by sun protection and regular dermatological inspection.

PMID:
16281625
[Indexed for MEDLINE]
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