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Arch Dermatol. 2004;140:1001-1002.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

In this issue of the ARCHIVES, Nguyen and colleagues present a clinically oriented study of 24 consecutive patients with Proteus syndrome who were evaluated at the National Institutes of Health in Bethesda, Md.¹ The diagnostic criteria of Proteus syndrome proposed by an international working group some years ago were met in all cases.² Because this multisystem birth defect occurs rarely, an analysis of such a large cohort is most welcome.

ADDITIONAL EVIDENCE OF MOSAICISM

Nguyen and colleagues provide further support to the idea that the disorder is caused by a mutation that is lethal except in mosaicism. All cases were sporadic. A hierarchical cluster analysis showed that none of the cutaneous protean abnormalities was tightly linked to one extracutaneous defect or the other. This randomness of findings is best explained by mosaicism. On the other hand, the authors document a positive correlation between the number of different types of cutaneous lesions and the number . . . [Full Text of this Article]

THE IMPORTANCE OF SKIN SIGNS

NEW ELATTOPROTEUS LESIONS

THE PTEN STORY NOW COMES TO AN END

PRACTICAL ASPECTS
Rudolf Happle, MD

RELATED ARTICLE

Cutaneous Manifestations of Proteus Syndrome: Correlations With General Clinical Severity
Diem Nguyen, Joyce T. Turner, Cara Olsen, Leslie G. Biesecker, and Thomas N. Darling
Arch Dermatol. 2004;140(8):947-953.
ABSTRACT | FULL TEXT