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. 2010 Nov;63(5):799-804.
doi: 10.1016/j.jaad.2009.12.012. Epub 2010 Aug 14.

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

Thomas M Beachkofsky  1 , Julie C Sapp, Leslie G Biesecker, Thomas N Darling
Affiliations

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

Thomas M Beachkofsky et al. J Am Acad Dermatol. 2010 Nov.

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.

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Conflict of interest statement

Conflict of Interest Disclosure: The authors have no conflict of interest to declare.

Figures

Figure 1
Figure 1
Progression of a cerebriform connective tissue nevus early in childhood. Pictures of the left sole (A–D) and right sole (E–H) of a patient with Proteus syndrome were taken at ages 3.7 (A, E), 4.5 (B, F), 5.5 (C, G), and 7.5 (D, H) years. A plaque on the left sole expanded and new plaques appeared near the ball of foot and instep of the right sole.
Figure 2
Figure 2
Progression of a cerebriform connective tissue nevus later in childhood. Pictures of the left sole (A–C) and right sole (D–F) were taken of a patient with Proteus syndrome at ages 11.4 (A, D), 12.1 (B, E), and 13.4 (C, F) years.
Figure 3
Figure 3
Graphical analysis of interval changes in CCTN. The total CCTN score at different ages are plotted for each patient in the longitudinal study. The CCTN scores for the patients in Figures 1 and 2 are represented by a dotted line with open triangles and a dashed line with open squares, respectively.
See this image and copyright information in PMC

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