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Basal cell carcinomas, multiple milia of face and limbs, increased sweating and facial pigmentation, and sparse scalp and body hair.

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Incidence is not known. Inheritance seems to be either autosomal dominant or X-linked dominant.

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Main features are sparse and coarse scalp hair, sparse body hair, and multiple, often big milia on face and limbs, which tend to disappear around puberty. Basal cell carcinomas (most often multiple) develop in early adult life. Some patients complain about excessive sweating. Some researchers believe it is the same entity as Bazex syndrome.

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Atropine and other parasympatholytic drugs may reduce the excessive sweating, which could otherwise render taping for the endotracheal tube, the intravenous cannula, and ECG electrodes challenging. However, temperature must be controlled to prevent hyperthermia.

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Bazex Syndrome: X-linked dominant inherited disorder characterized by follicular atrophoderma in combination with hypotrichosis, hypohidrosis, and nevoid basal cell carcinoma.

Oley CA, Sharpe H, Chenevix-Trench G: Basal cell carcinomas, coarse sparse hair, and milia. Am J Med Genet 43:799, 1992.  [PubMed: 1642265]
Vabres P, de Prost Y: Bazex-Dupre-Christol syndrome: A possible diagnosis for basal cell carcinomas, coarse sparse hair, and milia [letter]. Am J Med Genet 45:786, 1993.  [PubMed: 8456866]

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