• 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.
(Arch Dermatol. 1989;125:1109-1114)
Samlaska CP, Levin SW, James WD, Benson PM, Walker JC, Perlik PC. Proteus Syndrome. Arch Dermatol. 1989;125(8):1109–1114. doi:10.1001/archderm.1989.01670200085015
Customize your JAMA Network experience by selecting one or more topics from the list below.