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Journal of the American Academy of Dermatology
Volume 63, n° 5
pages 799-804 (novembre 2010)
Doi : 10.1016/j.jaad.2009.12.012
accepted : 13 December 2009
Original Articles

Progressive overgrowth of the cerebriform connective tissue nevus in patients with Proteus syndrome
 

Thomas M. Beachkofsky, CAPT, USAF, MC, FS a, Julie C. Sapp, MS, CGC b, Leslie G. Biesecker, MD b, Thomas N. Darling, MD, PhD a,
a Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 
b Genetic Disease Research Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 

Reprint requests: Thomas Darling, MD, PhD, Department of Dermatology, Uniformed Services University of Health Sciences, 4301 Jones Bridge Rd, Bethesda, MD 20814.
Abstract
Background

Proteus syndrome is a rare overgrowth disorder that almost always affects the skin.

Objective

Our purpose was to evaluate progression of skin lesions in patients with Proteus syndrome.

Methods

Skin findings were documented in 36 patients with Proteus syndrome. Progression of skin lesions in 16 of these patients was assessed by comparing photographs obtained on repeated visits for an average total duration of 53 months.

Results

The skin lesion most characteristic of Proteus syndrome, the cerebriform connective tissue nevus, showed progression in 13 children but not in 3 adults. The cerebriform connective tissue nevus progressed by expansion into previously uninvolved skin, increased thickness, and development of new lesions. Lipomas increased in size, number, or both in 8 of 10 children with lipomas. In contrast, epidermal nevi and vascular malformations generally did not spread or increase in number.

Limitations

Only 3 adults with Proteus syndrome were evaluated longitudinally.

Conclusion

The cerebriform connective tissue nevus in Proteus syndrome grows throughout childhood but tends to remain stable in adulthood.

The full text of this article is available in PDF format.

Key words : cerebriform connective tissue nevus, overgrowth, progression, Proteus syndrome



 Supported by Sulzberger Laboratory for Dermatologic Research, and the Intramural Research Program of the National Institutes of Health, National Human Genome Research Institute.
 Conflicts of interest: None declared.



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