| Bazex-Dupré-Christol syndrome in a 1-year-old boy and his mother. | |
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MedLine Citation:
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PMID: 18304168 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Bazex-Dupré-Christol syndrome is a rare genodermatosis with cancer predisposition, characterized by follicular atrophoderma, multiple milia, congenital hypotrichosis, hypohidrosis and basal cell malformations that include nevoid basal cell carcinomas of early onset. We present two patients with this syndrome, a 1-year-old boy with diffuse scalp and eyebrows alopecia, milia papules on the face, ears, trunk, and limbs. Hypohidrosis was observed on his trunk and head. His 16-year-old mother had identical changes since childhood, with hair fragility, and multiple atrophic "ice pick" follicular depressions on the dorsa of her hands. She also had a basal cell carcinoma on her face. Microscopic examination of hairs from the mother revealed abnormalities such as diameter irregularities, broken shafts, trichorrexis nodosa and pili bifurcatti. Pili bifurcatti is an uncommon hair shaft dysplasia that has not before been observed in Bazex-Dupré-Christol syndrome. |
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Authors:
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Andrezza Camarinha Napolitano Barcelos; Marcello Menta Simonsen Nico |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pediatric dermatology Volume: 25 ISSN: 1525-1470 ISO Abbreviation: Pediatr Dermatol Publication Date: 2008 Jan-Feb |
Date Detail:
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Created Date: 2008-02-28 Completed Date: 2008-04-07 Revised Date: 2009-03-03 |
Medline Journal Info:
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Nlm Unique ID: 8406799 Medline TA: Pediatr Dermatol Country: United States |
Other Details:
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Languages: eng Pagination: 112-3 Citation Subset: IM |
Affiliation:
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Department of Dermatology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil. zza@ig.com.br |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acrodermatitis
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diagnosis,
genetics Adolescent Basal Cell Nevus Syndrome / diagnosis, genetics* Carcinoma, Basal Cell / diagnosis, pathology* Cell Transformation, Neoplastic Female Heterozygote* Humans Hypohidrosis / diagnosis, genetics Hypotrichosis / diagnosis, genetics Infant Male Mothers Pedigree Precancerous Conditions / pathology* Prognosis Skin Neoplasms / diagnosis, genetics* Syndrome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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