The irregular, flat areas of increased skin pigment in McCune-Albright syndrome are called cafe-au-lait spots, or macules (CALM), because in children with light complexions they are the color of coffee with milk. In dark-skinned individuals these spots may be difficult to see. Most children have the pigment from birth, and it almost never becomes more extensive. The pattern of the pigment distribution is unique, often starting or ending abruptly at the midline on the abdomen in front or at the spine in back. Some children have no cafe-au-lait pigment at all; in a few, it is confined to small areas, such as the nape of the neck or crease of the buttocks. Most commonly the patches are on the buttocks and lumbosacral back. The patches are usually large, and have irregular borders resembling the "coast of Maine". (See picture) This is in contrast to the spots of neurofibromatosis, which have a smooth border resembling the "coast of California".
     Pam's Note: To me, these spots should send out a bit of a warning signal. Had I known that what I thought was a cute, albeit extensive, birthmark on Hannah indicated any type of syndrome, we could have avoided letting things get out of hand simply because we didn't know anything was wrong. I think if your child has these markings, that you should have a complete checkup and blood work to see what is going on. I've wished a million times since we found out Hannah has MAS that our pediatrician had known what the cafe-au-lait spots signify. 

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