Progressive overgrowth of the cerebriform connective tissue nevus in patients with Proteus syndrome - 24/04/13
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.
Le texte complet de cet article est disponible en PDF.Key words : cerebriform connective tissue nevus, overgrowth, progression, Proteus syndrome
Plan
| Supported by Sulzberger Laboratory for Dermatologic Research, and the Intramural Research Program of the National Institutes of Health, National Human Genome Research Institute. |
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| Conflicts of interest: None declared. |
Vol 63 - N° 5
P. 799-804 - novembre 2010 Retour au numéro
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