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


CONCLUSIONS
Only 3 adults with Proteus syndrome were evaluated longitudinally.


CONCLUSIONS
The cerebriform connective tissue nevus in Proteus syndrome grows throughout childhood but tends to remain stable in adulthood.
Beachkofsky
Thomas M
TM
Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814, USA.
Sapp
Julie C
JC
Biesecker
Leslie G
LG
Darling
Thomas N
TN
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D003238:Connective Tissue / Q000254:growth & development / Q000473:pathology*; D018450:Disease Progression; D004817:Epidermis / Q000254:growth & development / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009506:Nevus / Q000473:pathology*; D016715:Proteus Syndrome / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*; D055815:Young Adult