Proteus syndrome: A rare cause of gigantic limb

A congenital disorder with variable manifestations, including partial gigantism of the hands and feet with hypertrophy of soles, nevi, hemihypertrophy, gynecomastia, macrocephaly and other skull abnormalities, and abdominal lipomatosis. The cause is unknown, although a genetic origin, generally of a...

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Bibliographic Details
Main Authors: Nandini Chakrabarti, Chandan Chattopadhyay, Majhi Bhuban, Salil Kumar Pal
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2014-01-01
Series:Indian Dermatology Online Journal
Subjects:
Online Access:http://www.idoj.in/article.asp?issn=2229-5178;year=2014;volume=5;issue=2;spage=193;epage=195;aulast=Chakrabarti
Description
Summary:A congenital disorder with variable manifestations, including partial gigantism of the hands and feet with hypertrophy of soles, nevi, hemihypertrophy, gynecomastia, macrocephaly and other skull abnormalities, and abdominal lipomatosis. The cause is unknown, although a genetic origin, generally of autosomal-dominant transmission, has been conjectured. Symptoms can be treated, but there is no known cure. We present the case of a young male with grotesque overgrowth of the right lower limb, splenomegaly and multiple nevi. Angiography revealed venous malformation within the limb. The findings are in conformity to the criteria for the Proteus syndrome.
ISSN:2229-5178