| LEOPARD syndrome: what are café noir spots? | |
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MedLine Citation:
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PMID: 18789084 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retarded growth, and deafness syndrome (multiple lentigines syndrome) is most often characterized by multiple lentigines and cardiac conduction defects. Café noir spot is a term proposed, by analogy to café au lait spots, for the larger and darkly pigmented patches that are frequently observed in patients with this syndrome. Although presumed by some authors to represent lentigines, the histologic features of café noir spots have not been well documented in the literature. Only two previous cases have been reported in which a biopsy of the café noir spots than melanocytic nevi. We describe the histologic characteristics of seven café noir spots in six patients with lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retarded growth, and deafness syndrome. Three lesions represented melanocytic nevi (one with dysplastic features), and four were compatible with lentigo simplex. These findings help our understanding of the histologic spectrum of pigmented lesions in lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retarded growth, and deafness syndrome. |
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Authors:
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Alfonso Rodríguez-Bujaldón; Carmen Vazquez-Bayo; Rafael Jimenez-Puya; Manuel Galan-Gutierrez; José Moreno-Gimenez; Alfonso Rodriguez-Garcia; Jesus Tercedor; Antonio Velez-Garcia |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Pediatric dermatology Volume: 25 ISSN: 1525-1470 ISO Abbreviation: Pediatr Dermatol Publication Date: 2008 Jul-Aug |
Date Detail:
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Created Date: 2008-09-15 Completed Date: 2009-02-06 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: 444-8 Citation Subset: IM |
Affiliation:
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Service of Dermatology, Universitary Hospital Reina Sofía, Córdoba, Spain. alfonsobujaldon@yahoo.es |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Child Female Humans LEOPARD Syndrome / pathology* Lentigo / pathology Male Middle Aged Nevus, Pigmented / pathology Skin / pathology Skin Pigmentation* |
| Comments/Corrections | |
Erratum In:
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Pediatr Dermatol. 2009 Jan-Feb;26(1):127 Note: Vazquez-Bayo, Carmen [added]; Jimenez-Puya, Rafael [added]; Galan-Gutierrez, Manuel [added]; Moreno-Gimenez, José [added]; Rodriguez-Garcia, Alfonso [added];Tercedo, Jesus [added]; Velez-Garcia, Antonio [added]. |
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