Bazex-Dupre-Christol syndrome – A memorable family


  • Rabia Ghafoor
  • Muhammad Irfan Anwar
  • Moizza Tahir


Basal cell carcinoma, hypotrichosis, follicular atrophoderma


Bazex-Dupre-Christol syndrome (BDCS) is clinically characterized by multiple basal cell carcinomas of the face occurring mainly during the second and third decade of life, follicular atrophoderma predominantly of the dorsum of hands and feet and generalized hypotrichosis, sometimes with pili torti and trichorrhexis nodosa. Common associated features are milia, hypohidrosis and calcifying epithelial tumours. We herein report four members of one family with BDC syndrome, a mother and her three daughters. In the mother and one daughter, the clinical picture was very typical with all major features, whereas in the others only follicular atrophoderma, milia and hypotrichosis were present. BDCS is a hereditary multiple BCC syndrome whose pattern of inheritance is thought to be X-linked dominant, which implies that all daughters of the affected mothers should have this disease. In this case three out of four daughters are suffering from BDCS but one is totally asymptomatic. To our knowledge, this is the first report of a family with BDC from Pakistan. 


Bazex A, Dupre A, Christol B. Genodermatose complexe de type indetermine associant une hypotrichose, un état atrophodermique generalise et des degenerescences cutanees multiples (epitheliomas-basocellulaires). Bull Soc Dermatol Syphiligr. 1964;71:206.

Bazex A, Dupre A, Christol B. Atrophodermic folliculaire, proliférations baso-cellulaires et hypotrichose. Ann Dermatol Syphiligr. 1966;93:241-54.

Vabres P, Lacombe D, Rabinowitz LG et al. The gene for Bazex-Dupre-Christol syndrome maps to chromosome Xq. J Invest Dermatol. 1995;105:87-91.

Kidd A, Carson L, Gregory DW et al. A Scottish family with Bazex-Dupre-Christol syndrome: follicular atrophoderma, congenital hypotrichosis, and basal cell carcinoma. J Med Genet. 1996;33:493-7.

Glaessl A, Hohenlautner U, Landthaler M et al. Sporadic Bazex-Dupre-Christol-like syndrome: early onset basal cell carcinoma, hypohidrosis, hypotrichosis, and prominent milia. Dermatol Surg. 2000;26:152-4.

Andreani V, Richard M, Folchetti G et al. Congenital hypotrichosis and milia with spontaneous regression during adolescence or Oley syndrome: a variant of Bazex- Dupre-Christol syndrome. Ann Dermatol Venereol. 2000;127:285-8.

Goeteyn M, Geerts ML, Kint A et al. The Bazex-Dupre-Christol syndrome. Arch Dermatol. 1994;130:337-42.

Colomb D, Ducros B, Boussuge N. Bazex, Dupre and Christol syndrome. Apropos of a case with prolymphocytic leukemia. Ann Dermatol Venereol. 1989;116:381-7.

Glaessl A, Hohenlautner U, Landthaler M et al. Sporadic Bazex-Dupre-Christol-like syndrome: early onset basal cell carcinoma, hypohidrosis, hypotrichosis, and prominent milia. Dermatol Surg. 2000;26:152-4.

Maleville J, Alt J, Grosshans E. Atrophodermie folliculaire, pseudo-pelade, kératose pilaire et état ichthyosique. Bull Soc Dermatol Syphiligr. 1969;76:85-6.

Sourreil P, Verger P, Beylot C et al. Atrophodermie folliculaire familiale mixte. Bull Soc Dermatol Syphiligr. 1968;75:116-7.

Meynadier J, Guilhou JJ, Barneon G et al. Follicular atrophoderma, hypotrichosis, and multiple milia associated with minimal osteo-cartilaginous dystrophies. Familial study of three cases. Ann Dermatol Venereol. 1979;106:497-501.

Tuzun Y, Mat MC, Serdaroglu S et al. Follicular atrophoderma with scrotal tongue. Pediatr Dermatol. 1987;4:328-31.

Herges A, Stieler W, Stadler R. Bazex-Dupre-Christol syndrome. Follicular atrophoderma, multiple basal cell carcinomas and hypotrichosis. Hautarzt. 1993;44:385-91.

Michaelsson G, Olsson E, Westermark P. The Rombo syndrome: a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. Acta Derm Venereol. 1981;61:497-503.

Inoue Y, Ono T, Kayashima K et al. Hereditary perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts. Br J Dermatol 1998;139:713-8.

Vabres P, de Prost Y. Bazex-Dupre-Christol syndrome: a possible diagnosis for basal cell carcinomas, coarse sparse hair, and milia. Am J Med Genet. 1993;45:786.






Case Reports