These authors contributed equally to this manuscript.
Multiple familial and pigmented basal cell carcinomas in early childhood – Bazex–Dupré–Christol syndrome
Article first published online: 24 MAR 2011
© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology
Journal of the European Academy of Dermatology and Venereology
Volume 26, Issue 1, pages 117–121, January 2012
How to Cite
Abuzahra, F., Parren, L.J.M.T. and Frank, J. (2012), Multiple familial and pigmented basal cell carcinomas in early childhood – Bazex–Dupré–Christol syndrome. Journal of the European Academy of Dermatology and Venereology, 26: 117–121. doi: 10.1111/j.1468-3083.2011.04048.x
Funding support This study was not supported.
Conflict of interest None declared.
- Issue published online: 15 DEC 2011
- Article first published online: 24 MAR 2011
- Received: 8 November 2010; Accepted: 21 February 2011
Background Bazex–Dupré–Christol syndrome (BDCS) is an X-linked dominantly inherited disorder affecting hair follicle structures. Currently, hypotrichosis, follicular atrophoderma and basal cell carcinomas are considered frequent symptoms of the disorder whereas milia are supposed to reflect infrequent clinical signs. Usually, basal cell carcinomas in this disease manifest from the second decade of life onwards.
Case report Here, we studied a novel multigeneration family of German origin with BDCS. Interestingly, two family members developed pigmented basal cell carcinomas in early childhood, at the age of 3 and 5 years, respectively. The differentiation from other pigmented lesions was accomplished by both dermoscopy and histopathology. A thorough survey of the current literature revealed that milia were present in almost all patients with BDCS reported, as is the case in our family.
Conclusions We suggest that milia should also be considered frequent symptoms in BDCS. For the first time, to the best of our knowledge, we describe the occurrence of pigmented basal cell carcinomas in BDCS during the first decade of life. Our observation emphasizes the importance of screening for cutaneous malignancies in this disorder already at young age.