BAZEX-DUPRÉ-CHRISTOL SYNDROME

By Gary M. White, MD


Bazex-Dupré-Christol syndrome (BDCS) is an X-linked dominantly inherited disorder characterized by congenital hypotrichosis, hypohidrosis, follicular atrophoderma, multiple milia and basal cell carcinomas.

(As a reminder, X-linked dominant disorders do not necessarily affect males more than females. All daughters of an affected father will also be affected but none of his sons will be affected--unless the mother is also affected). In addition, the mother of an affected son is also affected.)

References

A 2 year old girl with congenital hypotrichosis if the scalp and eyebrows along with decreased sweat. Her 3 brothers mother and 3 maternal uncles had similar findings. Exam also showed multiple milia of the nose and cheeks. Arch Argent Pediatr 2015;113(5):e256-e259

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