| Phacomatosis pigmentovascularis: a new syndrome? Report of four cases. | |
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MedLine Citation:
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PMID: 3422849 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinically similar to those observed in the Sturge-Weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of a nevus flammeus in the Sturge-Weber syndrome; however, melanocytes of normal aspect were present in the middle and deep dermis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigmentovascularis seems appropriate for this apparently new neurocutaneous syndrome. |
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Authors:
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R Ruiz-Maldonado; L Tamayo; A M Laterza; G Brawn; A Lopez |
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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: 4 ISSN: 0736-8046 ISO Abbreviation: Pediatr Dermatol Publication Date: 1987 Nov |
Date Detail:
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Created Date: 1988-01-26 Completed Date: 1988-01-26 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: 189-96 Citation Subset: IM |
Affiliation:
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Department of Pediatric Dermatology, Instituto Nacional de Pediatria S.S.A., Mexico City, Mexico. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Brain Diseases
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complications Child, Preschool Eye Diseases / complications Female Hemangioma / complications* Humans Infant Male Pigmentation Disorders / complications* Skin Neoplasms / complications* Syndrome |
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