Mongolian Spots

September 14, 2005
Alexander K. C. Leung, MD

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.

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.

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