Proteus syndrome
C. P. Samlaska, S. W. Levin, W. D. James, P. M. Benson, J. C. Walker and P. C. Perlik
Department of Pediatrics, Walter Reed Army Medical Center, Washington, DC 20307-5001.
The term Proteus syndrome was coined in 1983 to describe a disorder of
skeletal, hamartomatous, and other mesodermal malformations. The syndrome
was named after the Greek god Proteus, whose name means "the Polymorphous."
Clinical features of this new syndrome are currently being defined.
Including the case reported herein, we have found 34 patients with Proteus
syndrome described in the English literature. Major clinical findings,
defined as those findings seen in more than half of the cases, include
hemihypertrophy, macrodactyly, exostoses, epidermal nevi, characteristic
cerebriform masses involving the plantar or palmar surfaces, a variety of
subcutaneous masses, and scoliosis. Histologic examination of subcutaneous
masses has identified a variety of lipomatous, hamartomatous, and
angiomatous tumors.
