Skin melanocytic tumor
Lentigines, melanotic macules and melanocytic hyperplasia
Café-au-lait spot
Author: Christopher S. Hale, M.D.
Last author update: 1 April 2013
Last staff update: 24 September 2024
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Café-au-lait spot
Table of Contents
Definition / general | Terminology | Epidemiology | Case reports | Clinical images | Microscopic (histologic) description | Electron microscopy description | Differential diagnosisCite this page: Hale CS. Café-au-lait spot. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumormelanocyticcafeaulait.html. Accessed November 27th, 2024.
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)
Terminology
- Café au lait is French for "coffee with milk", referring to light brown color of lesions
- Often abbreviated as "CALM"
Epidemiology
- 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
- 7 week old infant with McCune-Albright syndrome and multiple, bilateral lesions (Pediatr Dermatol 1991;8:35)
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
- Becker melanosis: increased hair and smooth muscle in lesion
- Ephelides (freckles)
- Lentiginous nevus
- Mongolian spot: blue pigmentation, larger
- Pigmented nevus: deeper pigment clinically, melanocytes not restricted to basal epidermis
