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Grand Rounds |

The Multifaceted Challenges of Proteus Syndrome

Leslie G. Biesecker, MD
JAMA. 2001;285(17):2240-2243. doi:10.1001/jama.285.17.2240.
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Proteus syndrome is a rare and sporadic disorder that causes postnatal overgrowth of multiple tissues in a mosaic pattern. The overgrowth can involve skin, subcutaneous tissue, connective tissue (including bone), the central nervous system, and viscera. Complications of Proteus syndrome include, among others, progressive skeletal deformities, invasive lipomas, benign and malignant tumors, and deep venous thrombosis with pulmonary embolism. Care of patients with Proteus syndrome presents significant challenges to both physicians and parents because of the various medical as well as psychosocial consequences of the disease. Herein, the case of a 5-year-old patient who manifested a number of these complications is presented. Current knowledge about the diagnosis, natural history, etiology, and management of the disorder is reviewed.

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Figure 1. Left Foot Hyperplasia at Ages 3 and 5 Years
Graphic Jump Location
A, Sole of the left foot at age 3 years. Note the distortion of the toes due to overgrowth and the hyperplasia with deep grooves of the forefoot (cerebriform hyperplasia or connective tissue nevus). B, Progression of the hyperplasia of the sole at age 5 years.
Figure 2. Knee Overgrowth at Ages 3 and 5 Years
Graphic Jump Location
A, Lateral view of the legs showing early sign of hyperplastic overgrowth at the knees at age 3 years. B, At 5 years of age, the knee overgrowth has progressed and a fixed flexion deformity of the ankle is now apparent.
Figure 3. Hand Overgrowth at Age 3 Years
Graphic Jump Location
At 3 years, the thumb and index and middle fingers of the left hand show significant overgrowth.
Figure 4. Radiographic Findings at Age 5 Years
Graphic Jump Location
A, Ectopic calcification (arrow) in the quadriceps and the outline of the fixed mass surrounding the knee with partial calcification (delineated by arrowheads). B, Fixed position of the foot and partial calcification (arrow) within this mass (arrowhead). C, Irregular overgrowth of the phalanges of digits 1 through 3 of the left hand.

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