Derm

Cafe-Au-Lait Macule

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Cafe-Au-Lait Macule, Cafe-Au-Lait Spot, Cafe Au Lait

  • Pathophysiology
  1. Congenital or develops in early childhood
  2. Hyperpigmentation from increased Melanin deposition
  • Epidemiology
  1. Often present at birth
  2. Isolated lesions are common (present in up to 10-30% of the population)
  • Symptoms
  1. Asymptomatic
  • Signs
  1. "Coffee with milk" (tan to brown) colored flat Macule
  2. Usually located on trunk
  3. Size varies from <4 cm in infants to 20-30 cm in adults
  • Red Flags
  • Criteria to consider associated congenital conditions below
  1. Six or more lesions that meet size criteria
  2. Size criteria
    1. Prepubertal: Lesions 5 mm or larger
    2. Postpubertal: Lesions 15 mm or larger
  • Associated Conditions (if >6 lesions)
  1. Neurofibromatosis
  2. Tuberous Sclerosis
  3. Albright Syndrome
  4. Fanconi Anemia
  • Management
  1. None needed
  2. Consider treatment only for cosmetic reasons
    1. Laser therapy
    2. Surgical excision
  • References
  1. Plensdorf (2017) Am Fam Physician 96(12): 797-804 [PubMed]
  2. Plensdorf (2009) Am Fam Physician 79(2): 109-16 [PubMed]