II. Pathophysiology

  1. Congenital or develops in early childhood
  2. Hyperpigmentation from increased Melanin deposition

III. Epidemiology

  1. Often present at birth
  2. Isolated lesions are common (present in up to 10-30% of the population)

IV. Symptoms

  1. Asymptomatic

V. 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

VI. 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

VII. Associated Conditions (if >6 lesions)

  1. Neurofibromatosis
  2. Tuberous Sclerosis
  3. Albright Syndrome
  4. Fanconi Anemia

VIII. Management

  1. None needed
  2. Consider treatment only for cosmetic reasons
    1. Laser therapy
    2. Surgical excision

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