Skin melanocytic tumor

Lentigines, melanotic macules and melanocytic hyperplasia

Café-au-lait spot

Last author update: 1 April 2013
Last staff update: 8 November 2021

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PubMed Search: Café-au-lait spot

Christopher S. Hale, M.D.
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Cite this page: Hale CS. Café-au-lait spot. website. Accessed February 6th, 2023.
Definition / general
  • Pigmented macule 0.5 mm+, flat, round / oval, sharply demarcated with even pigmentation; long axis is along cutaneous nerve tract
  • May have irregular borders
  • Pigment histologically restricted to basal layer of epidermis
  • Usually present at birth (Wikipedia)
  • Café au lait is French for "coffee with milk", referring to light brown color of lesions
  • Often abbreviated as "CALM"
  • If solitary, usually nonsyndromic
  • In U.S. study of children under 5 years, 19% had one, 0.75% had > 2; more present in African Americans (Arch Dis Child 1966;41:316)
  • If solitary, usually nonsyndromic; multiple associated with neurofibromatosis type 1 - any person with 6+ lesions should be presumed to have neurofibromatosis until proven otherwise
  • Also associated with McCune-Albright syndrome and other syndromes (eMedicine)
Case reports
Clinical images

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Various images

Various images

Axillary freckling

Lesion of face

Neurofibromatosis type I patient

Microscopic (histologic) description
  • Basal hyperpigmentation of epidermis, no deeper pigmentation
  • Increased amount of pigment in melanocytes, some have giant pigment granules (Tohoku J Exp Med 1976;118:255)
  • Melanophages are rare
  • Adnexal epithelium has no increased pigment
  • Syndromic patients: more DOPA+ melanocytes in lesional skin contrasted to sporadic patients with decreased DOPA+ melanocytes within lesion (Arch Dermatol 1970;102:442)
Electron microscopy description
  • Macromelanosomes
Differential diagnosis
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