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Bazex syndrome

MIM.301845

Thursday 6 January 2011

Bazex syndrome is an X-linked dominant disorder characterized by a triad of congenital hypotrichosis, follicular atrophoderma affecting the dorsa of the hands and feet, the face, and extensor surfaces of the elbows or knees, and the development of basal cell neoplasms, including basal cell nevi and basal cell carcinomas from the second decade onward.

References

- Yung, A., Newton-Bishop, J. A. A case of Bazex-Dupre-Christol syndrome associated with multiple genital trichoepitheliomas. Brit. J. Derm. 153: 664-699, 2005. [PubMed: 16120163]

- Vabres, P., Lacombe, D., Rabinowitz, L. G., Aubert, G., Anderson, C. E., Taieb, A., Bonafe, J.-L., Hors-Cayla, M.-C. The gene for Bazex-Dupre-Christol syndrome maps to chromosome Xq. J. Invest. Derm. 105: 87-91, 1995. [PubMed: 7615983]