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©2013 UpToDate ® Print Email Cafe-au-lait spots in McCune-Albright syndrome A) A typical lesion on the face, chest, and arm of a 5-year-old girl with McCune-Albright syndrome, which demonstrates jagged "coast of Maine" borders and the tendency for the lesions to both respect the midline and follow the developmental lines of Blaschko (a configuration of skin lesions characterized by arcs on the upper chest, S shapes on the abdomen, and V shapes over the posterior midline, caused by patterns of x-chromosome inactivation). B) Typical lesions that are often found on the nape of the neck and crease of the buttocks are shown (arrows). Reproduced from: Dumitrescu CE, Collins MT. McCune-Albright syndrome. Orphanet J Rare Dis 2008; 3:12. Copyright ©2008 BioMed Central Ltd.