Document Detail


Proteus syndrome: report of the first Japanese case with special reference to differentiation from Klippel-Trenaunay-Weber syndrome.
MedLine Citation:
PMID:  1328340     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
This is the first report of a Japanese girl with Proteus syndrome. She presented with growth acceleration and precocious development of the left breast as well as macrodactyly, hemihypertrophy, a subcutaneous preaxillary mass, portwine stains, connective tissue nevi, and a depigmented macule. All these abnormalities were confined to the left side of her body. Although most of the manifestations fit those of Proteus syndrome, the presence of the portwine stains and hemihypertrophy also suggested Klippel-Trenaunay-Weber syndrome. The findings in our patient suggest that the most important characteristic distinguishing Proteus syndrome from Klippel-Trenaunay-Weber syndrome is the presence of functional abnormalities such as a growth spurt and precocious breast development. Proteus syndrome may be genetically different from the Klippel-Trenaunay-Weber syndrome.
Authors:
Y Hagari; M Aso; S Shimao; T Okano; A Kurimasa; K Takeshita
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of dermatology     Volume:  19     ISSN:  0385-2407     ISO Abbreviation:  J. Dermatol.     Publication Date:  1992 Aug 
Date Detail:
Created Date:  1992-11-18     Completed Date:  1992-11-18     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7600545     Medline TA:  J Dermatol     Country:  JAPAN    
Other Details:
Languages:  eng     Pagination:  477-80     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Faculty of Medicine, Tottori University, Yonago, Japan.
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Diagnosis, Differential
Female
Humans
Klippel-Trenaunay-Weber Syndrome / diagnosis*
Proteus Syndrome / diagnosis*

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