Proteus syndrome: report of a case with recurrent abdominal lipomatosis.

[proteus syndrome]

Proteus syndrome (PS ) is an extremely rare congenital hamartomatous syndrome that was first delineated by Cohen and Hayden (1 ). The estimated prevalence is less than 1 per 1 ,000 ,000 live births (2 ). It is a sporadic disorder that causes overgrowth of multiple tissues , especially bone , fat , and other connective tissues in a patchy or mosaic pattern . Subcutaneous as well as internal lipomas that may grow to an enormous size are frequently observed . Nevertheless , among the internal lipomas , abdominal lipomatosis is rare (3 ), with less than 15 cases reported . Herein , we report the first patient described with this distinctive syndrome associated with lipomatosis involving the epiploon .