3 Iconography
Access to the text (HTML) Access to the text (HTML)
PDF Access to the PDF text

Access to the full text of this article requires a subscription.
  • If you are a subscriber, please sign in 'My Account' at the top right of the screen.

  • If you want to subscribe to this journal, see our rates

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.

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


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


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.


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.


Only 3 adults with Proteus syndrome were evaluated longitudinally.


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.

© 2009  American Academy of Dermatology, Inc.@@#104156@@
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Article Outline
You can move this window by clicking on the headline