| Phacomatosis pigmentokeratotica with nephroblastoma and juvenile hypertension. | |
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MedLine Citation:
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PMID: 20526546 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Phacomatosis pigmentokeratotica is characterized by the coexistence of an organoid epidermal naevus, follow-ing Blaschko's lines, and a large speckled lentiginous naevus, typically arranged in a chequerboard pattern. This entity has been isolated from the group of epidermal naevus syndromes and is frequently associated with extracutaneous anomalies. We report here the first observation of phacomatosis pigmentokeratotica associated with nephroblastoma. In addition to this paediatric renal tumour, the coexistence of juvenile arterial hypertension suggests an associated vascular defect. The link between the extracutaneous manifestations and cutaneous twin spot phenotype is discussed. |
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Authors:
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Simon Jacobelli; Stéphanie Leclerc-Mercier; Rémi Salomon; Olivier Hartmann; Frances Brunelle; Rudolf Happle; Christine Bodemer; Smail Hadj-Rabia |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Acta dermato-venereologica Volume: 90 ISSN: 1651-2057 ISO Abbreviation: Acta Derm. Venereol. Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-06-07 Completed Date: 2010-09-07 Revised Date: 2012-01-02 |
Medline Journal Info:
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Nlm Unique ID: 0370310 Medline TA: Acta Derm Venereol Country: Sweden |
Other Details:
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Languages: eng Pagination: 279-82 Citation Subset: IM |
Affiliation:
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Department of Dermatology, Necker Hospital, MAGEC Reference Centre, NSERM U-781, René Descartes Paris V University, Paris, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Antihypertensive Agents
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therapeutic use Chemotherapy, Adjuvant Genotype Humans Hypertension, Renovascular / drug therapy, etiology*, genetics Infant Kidney Neoplasms / etiology*, genetics, therapy Male Nephrectomy Neurocutaneous Syndromes / complications*, genetics, pathology Phenotype Renal Artery / abnormalities Skin / pathology* Treatment Outcome Wilms Tumor / etiology*, genetics, therapy |
| Chemical | |
Reg. No./Substance:
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0/Antihypertensive Agents |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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