Café au Lait Spots

These are hyperpigmented, regularly bordered, sharply demarcated macules that are usually tan or light brown in whites and dark brown in dark-skinned persons. The lesions are characterized by an increased number of melanocytes and an increased amount of melanin in the epidermis.

Solitary caf au lait spots are found in 10% of healthy children and have no clinical significance. However, the presence of five or more spots 0.5 cm or larger in a prepubertal child or six or more spots larger than 1.5 cm in a postpubertal child is virtually pathognomonic for neurofibromatosis type I (von Recklinghausen's disease). Caf au lait spots are also found with increased frequency in patients with Russell-Silver syndrome, tuberous sclerosis, Fanconi's anemia, Gaucher's disease, and Turner's syndrome.

FOR MORE INFORMATION:

•Leung AKC, Wong BE, Cho HY, Chan PY. Caf au lait spots. Emerg Med. 1997;29(10):30-35.

•Mautner VF, Lindenau M, Baser ME, et al. Skin abnormalities in neurofibromatosis. Arch Dermatol. 1997;133:1539-1543.

•Tanner M, Proksch E, Christophers E. Von Recklinghausen neurofibromatosis and dermal melanocytic nevi. Hautarzt. 1995;46:263-267.