[Miliaria and follicular atrophodermia as an early sign of Bazex-Dupré-Christol syndrome].
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.
Beljan
Gabriele
G
Klinik und Poliklinik für Hautkrankheiten des Universitätsklinikums Münster. beljan@uni-muenster.de
Metze
Dieter
D
D001284:Atrophy; D003937:Diagnosis, Differential; D018450:Disease Progression; D005148:Facial Dermatoses / Q000473:pathology*; D005260:Female; D006197:Hair / Q000002:abnormalities* / Q000473:pathology; D006801:Humans; D007007:Hypohidrosis / Q000473:pathology*; D007039:Hypotrichosis / Q000473:pathology*; D007223:Infant; D008883:Miliaria / Q000473:pathology*; D018295:Neoplasms, Basal Cell / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*; D013577:Syndrome