| iv> | | | | Café-au-lait spots are harmless. CAL spots are |
| As reflected by the name - which of course | | | | caused by an increase in melanin content with the |
| means "coffee with milk". They are usually | | | | presence of giant melanosomes. A significant |
| present at birth, but may arise later in the first | | | | increase in melanocyte density is noted in the |
| few years of life. They are much more common | | | | CAL spots of patients with NF1 compared with |
| in black infants. | | | | patients who have isolated. About 50% of |
| Café-au-lait spots are dark spots on the skin | | | | individuals with NF1 have a spontaneous mutation. |
| that look like birthmarks. Most children have the | | | | The high incidence of new mutations is thought to |
| pigment from birth, and it almost never becomes | | | | result from the large size of the gene, which |
| more extensive. The pattern of the pigment | | | | increases the likelihood of spontaneous mutations. |
| distribution is unique, often starting or ending | | | | CAL spots without NF1 involvement. About 95 |
| abruptly at the midline on the abdomen in front or | | | | percent of people with NF1 have café-au-lait |
| at the spine in back. Most commonly the patches | | | | spots. In fact, birthmarks that could be classified |
| are on the buttocks and lumbosacral back. The | | | | as café-au-lait spots often show up in people |
| patches are usually large, and have irregular | | | | who don't have NF1. A person will only have one |
| borders resembling the "coast of Maine". This is in | | | | or two such birthmarks. CAL spots are more |
| contrast to the spots of neurofibromatosis, which | | | | frequently observed in children of African |
| have a smooth border resembling the "coast of | | | | American race. Café-au-lait spots are usually |
| California". They are light to medium brown. | | | | present at birth, increasing in size and number |
| Café-au-lait spots range from about about five | | | | with age. CAL lesions do not require medical care. |
| millimeters to more than several centimeters in | | | | Monitoring of associated conditions is required. |