Bazex-Dupré-Christol syndrome (BDCS)
نویسندگان
چکیده
منابع مشابه
A Scottish family with Bazex-Dupré-Christol syndrome: follicular atrophoderma, congenital hypotrichosis, and basal cell carcinoma.
Bazex-Dupre-Christol syndrome (BDCS) is an X linked dominant disorder of the hair follicle characterised by follicular atrophoderma, multiple basal cell carcinomas, hypotrichosis, milia, and localised hypohidrosis. Follicular atrophoderma (FA) are follicular funnel shaped depressions, "ice pick marks", seen most commonly on the dorsum of the hands. We describe the first known Scottish family wi...
متن کاملA case of Bazex-Dupré-Christol syndrome associated with multiple genital trichoepitheliomas.
reasonable adult dose and, for this child, 1 mg kg. There are no available data concerning the likely absorption of corticosteroid from a topical preparation such as Eumovate in a child of this age; however, it would be a coincidence if this event were not involved in the development of GPP in this case. While unlikely to be a common occurrence in everyday clinical practice it does appear that ...
متن کاملBazex-Dupre-Christol syndrome: A case report
Bazex-Dupre-Christol (BDC) syndrome is clinically characterizedby multiple basal cell carcinomas of the face that mainly occurduring the second and third decade of life, follicular atrophodermapredominantly of the dorsum sides of the hands and feetand generalized hypotrichosis; sometimes with pili torti andtrichorrhexis nodosa. Features commonly associated with BDCare milia, hypohidrosis and ca...
متن کاملCancer Prone Disease Section
Inheritance Bazex-Dupré-Christol syndrome is an X-linked dominant genodermatosis, with a prevalence below 1/1 000 000. The female to male ratio reaches the expected 2:1, and females appear to be less affected than males. There is intrafamilial and interfamilial phenotypic variability (Lacombe and Taïeb, 1995; Kidd et al., 1996). Most cases are European patients. Oley syndrome (Oley et al., 1992...
متن کاملBazex Syndrome*
Acrokeratosis Paraneoplastica or Bazex syndrome is a dermatologic manifestation usually associated with the diagnosis of squamous cell carcinoma of the upper aerodigestive tract. We report a case with exuberant clinical manifestations, exemplifying the typical cutaneous lesions in this rare syndrome, in a patient with squamous cell carcinoma of the esophagus.
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ژورنال
عنوان ژورنال: Atlas of Genetics and Cytogenetics in Oncology and Haematology
سال: 2013
ISSN: 1768-3262
DOI: 10.4267/2042/48768