Document Detail


Phakomatosis pigmentovascularis IIb with renal anomaly.
MedLine Citation:
PMID:  10671974     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Phakomatosis pigmentovascularis (PPV) is a rare congenital naevoid syndrome; most case reports originate in Japan. The major clinical manifestations consist of coexisting extensive naevus flammeus and pigmentary naevus with or without systemic involvement. We report an 8-year-old Taiwanese boy, who was born with extensive naevus flammeus and other anomalies comprising persistent aberrant Mongolian spot-like pigmentary patches, leg-length discrepancy, pelvic obliquity, scoliosis and bilateral melanosis oculi bulbi. Further investigation also revealed agenesis of the right kidney. The cutaneous lesions remained unchanged over a 3-year follow-up period. Within the classification of PPV, this boy's disorder represents an example of PPV IIb. Right kidney agenesis, which has never been observed in PPV, may be an incidental finding.
Authors:
C Huang; P Lee
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical and experimental dermatology     Volume:  25     ISSN:  0307-6938     ISO Abbreviation:  Clin. Exp. Dermatol.     Publication Date:  2000 Jan 
Date Detail:
Created Date:  2000-05-17     Completed Date:  2000-05-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7606847     Medline TA:  Clin Exp Dermatol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  51-4     Citation Subset:  IM    
Affiliation:
Dermatological Department, Sin-Lau Christian Hospital, Tainan, Taiwan.
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MeSH Terms
Descriptor/Qualifier:
Child
Humans
Kidney / abnormalities*
Male
Neurocutaneous Syndromes / congenital*,  pathology
Nevus, Pigmented / congenital*,  pathology
Skin Neoplasms / congenital*,  pathology

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


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