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MAJ Curt P. Samlaska, MC; Sondra W. Levin, MD; LTC William D. James, MC; MAJ Paul M. Benson, MC; CPT John C. Walker, MC; MAJ Paul C. Perlik, MC

Arch Dermatol. 1989;125(8):1109-1114.

Abstract
• 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)

Author Affiliations
USA; USA; USA; USA; USA

From the Dermatology Service (Drs Samlaska, James, and Benson), Department of Pediatrics (Drs Levin and Walker), and Orthopedic Service (Dr Perlik), Walter Reed Army Medical Center, Washington, DC.

Footnotes
Accepted for publication April 10, 1989.

The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.

Reprint requests to Dermatology Service, Walter Reed Army Medical Center, Washington, DC 20307-5001 (Dr Samlaska).

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