Skin - Melanocytic tumor
Other pigmented lesions and disorders of pigmentation
Café-au-lait spot

Author: Christopher Hale, M.D. (see Authors page)

Revised: 24 February 2017, last major update April 2013

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Café-au-lait spot

Cite this page: Café-au-lait spot. website. Accessed December 11th, 2018.
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

Images hosted on other servers:

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