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.
- BDCS should be considered as a differential diagnosis in patients with early onset or familial basal cell carcinomas.
- In one report, the age of onset of BCCs was from 9-50 years of age. They seemed more common on the face.
- The hypohidrosis can be generalized of just limited to the face.
- Some authors have reported a "pinched" appearance to the nose.
(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.)
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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