A Scottish family with Bazex-Dupre-Christol syndrome: follicular atrophoderma, congenital hypotrichosis, and basal cell carcinoma.

Author:

Kidd A,Carson L,Gregory D W,de Silva D,Holmes J,Dean J C,Haites N

Publisher

BMJ

Subject

Genetics (clinical),Genetics

Reference34 articles.

1. Several authors have reported a number of different features ranging from completely normal follicles to widened, plugged, or lax follicles.2517 FA is also seen in chondrodysplasia punctata (Conradi-Hunerman syndrome), another X linked dominant disorder, and in association with keratosis palmoplantaris, keratosis follicularis, and hyperhidrosis palmoplantaris.26

2. A Scottish family with Bazex-Dupre-Christol syndrome The only report7 of BDCS occurring in association with other malignancies described a 10 year old girl who was diagnosed as having BDCS at the age of 5 and who developed prolymphocytic leukaemia at the age of 10. In our family, the sister of II-2 died of a sarcoma at the age of 38 and the mother of 11-2 died of cancer of the cervix at the age of 45, but we do not know if these women also had BDCS

3. Genodermatose complexe de type indetermine associant une hypotrichose, un etat atrophodermique generalise et des degenerescences cutanees multiples (epitheliomas-basocellulaires). Bull Soc Fr Dermatol Syph;Bazex, A.; Dupre, A.; Christol, B.,1964

4. Atrophodermic folliculaire, proliferations baso-cellulaires et hypotrichose;Bazex, A.; Dupre, A.; Christol, B.;Ann Dermatol Syph,1966

5. Atrophodermie folliculaire, proliferations baso-cellulaires et hypotrichose;Pierard, J.; Dhondt, F.; Geerts, M.L.;Arch Belg Dermatol,1966

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