1994
DOI: 10.1001/archderm.130.3.337
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The Bazex-Dupre-Christol syndrome

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Cited by 26 publications
(47 citation statements)
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“…Multiple basal cell carcinomas arising at a rather young age are reported in the literature as a cutaneous feature of rare hereditary syndromes characterized by various additional developmental defects [1, 2, 3]. In the present family, no additional cutaneous or extracutaneous anomalies were found.…”
Section: Discussionmentioning
confidence: 48%
See 1 more Smart Citation
“…Multiple basal cell carcinomas arising at a rather young age are reported in the literature as a cutaneous feature of rare hereditary syndromes characterized by various additional developmental defects [1, 2, 3]. In the present family, no additional cutaneous or extracutaneous anomalies were found.…”
Section: Discussionmentioning
confidence: 48%
“…In general, they appear sporadically and either as a solitary lesion or in a small number, with a predilection of sun-exposed areas. A tendency to develop multiple basal cell carcinomas at an early age is a characteristic feature of some rare hereditary disorders such as Gorlin syndrome [1], Rombo syndrome [2]and Bazex-Dupré-Christol syndrome [3]in which the skin tumors are associated with various other cutaneous or extracutaneous anomalies. Moreover, multiple basal cell carcinomas are sometimes observed as a corollary of familial basaloid follicular hamartomas [4]or familial multiple trichoepitheliomas [5].…”
Section: Introductionmentioning
confidence: 99%
“…In the described family skin tumors were not basal cell carcinomas, but trichoepitheliomas. Trichoepitheliomas are not common in BDCS but they have been previously described in other affected families [Viksnins and Berlin, ; Goeteyn et al, ; Kidd et al, ]. The microscopic hair abnormalities (pili torti and trichorrhexis nodosa) found in the proband are a significant finding in BDCS [Beylot et al, ; Meynadier et al, ; Colomb et al, ].…”
Section: Discussionmentioning
confidence: 98%
“…The absence of male‐to‐male transmission and the fact that almost all daughters of affected fathers are affected has indicated that BDCS is an X‐linked dominant disorder [Viksnins and Berlin, ; Goeteyn et al, ; Rapenaloro et al, ]. Intrafamilial phenotypic variability is common, with milder expression in females consistent with X‐inactivation observed [Goeteyn et al, ]. The causative gene has not been identified but there is evidence of linkage to the region Xq24‐q27 [Vabres et al, ].…”
Section: Discussionmentioning
confidence: 99%
“…BDCS occurs among males and females, however its severity across the two genders is controversial. For instance, signs and symptoms were documented to be more severe among male patients, yet Abuzahra et al . reported a severe phenotype in female patients compared to males of the same family.…”
Section: Introductionmentioning
confidence: 99%