Cafe-au-lait spot The phrase cafe-au-lait comes from the French word for milk-coffee. A single cafe-au-lait spot can be an isolated finding in otherwise normal individuals. Multiple cafe-au-lait spots often indicate the presence of neurofibromatosis type 1 but may also be seen in other diseases including McCune-Albright syndrome, tuberous sclerosis, and Fanconi anemia. Cafe-au-lait spots are hyperpigmented lesions that can vary in color from light brown to dark brown with smooth borders and having a size of 1.5 cm or more in adults and 0.5 cm or more in children. Cafe-au-lait macules Cafe au lait spots Cafe-au-lait macule UMLS:C0221263 HP:0005601 Birthmark SNOMEDCT_US:201281002 Cafe-au-lait spots HP:0000957 Flat light-brown mark on skin UMLS:C0265974 SNOMEDCT_US:51089004 HP:0007454 Hypermelanotic macule