Saturday, June 15, 2024
HomeDermatologyDermatologic Manifestations of Proteus Syndrome

Dermatologic Manifestations of Proteus Syndrome

Background

Proteus syndrome (PS) is a sporadically occurring hamartomatous disorder associated with irregular asymmetric overgrowth of multiple body tissues and cell lineages.
Most malformations in patients with Proteus syndrome have a mesodermal origin. Characteristic plurifocal overgrowths (partial or regional gigantism) can involve any structure of the body but most commonly involve the bone, connective tissue, and fat. Although some evidence of this syndrome was published in the medical literature as early as 1907, the modern medical description of the disease is attributed to Cohen and Hayden, who identified the syndrome in 1979.
In 1983, to stress the polymorphic nature of the clinical manifestations of the disorder, Wiedermann named it “Proteus syndrome” after the Greek god Proteus, who could change his shape at will to avoid capture.

Perhaps the most famous case of Proteus syndrome is that of Joseph Merrick, the “Elephant Man” described by Sir Frederick Treves in 1884, who was made famous by a stage play and movie of the same name. Although first thought to have neurofibromatosis, Merrick is now believed to have had Proteus syndrome. Preserved castings of his soles show cerebriform cutaneous hyperplasia, a characteristic finding in persons with Proteus syndrome.

Previous articleHaberland Syndrome
Next articlePostpartum Hemorrhage
RELATED ARTICLES
- Advertisment -

Most Popular