Document Detail


Phacomatosis pigmentokeratotica with nephroblastoma and juvenile hypertension.
MedLine Citation:
PMID:  20526546     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Simon Jacobelli; Stéphanie Leclerc-Mercier; Rémi Salomon; Olivier Hartmann; Frances Brunelle; Rudolf Happle; Christine Bodemer; Smail Hadj-Rabia
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta dermato-venereologica     Volume:  90     ISSN:  1651-2057     ISO Abbreviation:  Acta Derm. Venereol.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-06-07     Completed Date:  2010-09-07     Revised Date:  2012-01-02    
Medline Journal Info:
Nlm Unique ID:  0370310     Medline TA:  Acta Derm Venereol     Country:  Sweden    
Other Details:
Languages:  eng     Pagination:  279-82     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Necker Hospital, MAGEC Reference Centre, NSERM U-781, René Descartes Paris V University, Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Antihypertensive Agents / 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:
0/Antihypertensive Agents

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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