II. Epidemiology

  1. Prevalence: Up to 2-5% of newborns

III. Pathophysiology

  1. Neural crest Melanocytes and their precursors with disrupted migration

IV. Signs

  1. Brown to black lesions that are typically flat
    1. Can be elevated
    2. May thicken as children get older
  2. Size can vary from a few millimeters to several centimeters
    1. In some cases, may be very large, involving an entire region of the body
    2. Lesions increase in size with age
      1. Head lesions double in size with age
      2. Extremity and trunk lesions triple in size with age
  3. Hair is variably present
  4. Typically with uniform pigmentation
  5. Broad Distribution (trunk, extremiities, face)

V. Types: Speckled Lentiginous Nevus (Nevus Spilus)

  1. Variant of Congenital Nevi
    1. Hairless round to irregularly shaped brown nevus
    2. Larger nevus contains 1-3 mm dots of dark brown pigmentation (may be elevated)
    3. Diameter ranges from 1-20 cm
  2. Malignant transformation is uncommon but can occur
    1. Size of lesion directs management - follow the same protocols for Congenital Nevi based on size below
    2. Observe and biopsy or excise lesions if atypical

VI. Lab: Histology

  1. Localized to the lower Dermis

VII. Differential Diagnosis

VIII. Complications: Melanoma

  1. Small Congenital Nevi <1.5 cm (adult size) rarely progress to Melanoma
  2. Medium size lesions may develop Melanoma in up to 0.7% of cases
  3. Giant Congenital Nevi >20 cm (adult size) carry up to a 7% lifetime risk of Melanoma
    1. Half of Melanomas in Giant Congenital Nevi occur by age 3-5 years

IX. Management

  1. General
    1. Carefully document lesion locations and size
      1. Consider images with a ruler (or labeled diagrams)
  2. Large Congenital Melanocytic Nevi (Giant Congenital Nevi)
    1. Criteria
      1. Garment Nevi or Giant Congenital Nevi: >14 cm in infants (>20 cm in adults)
      2. Large Congenital Nevi: >12 cm head or >7 cm elsewhere in infants (>20 cm adults)
    2. Precautions
      1. As noted above, Melanoma development in Giant Congenital Nevi occurs before age 3-5 years in 50% of cases
      2. Excise these lesions as infants or young children (before age 3-5 years)
        1. However, lesion excision does not completely eliminate the malignancy risk
      3. Observation may miss transformation due to depth of nevus
    3. Protocol
      1. Excise lesion as soon as possible (or currettage during the first 2 weeks of life)
      2. Close observation for recurrence (excision does not eliminate risk completely)
  3. Medium Congenital Melanocytic Nevi
    1. Criteria: 0.5 to 7 cm in infants (1.5 to 20 cm in adults)
    2. Protocol
      1. Close observation by dermatology
      2. Consider Punch Biopsy for risk stratification
        1. Deeper dermal lesions may elude early detection of malignant transformation despite observation
  4. Small Congenital Melanocytic Nevi
    1. Criteria: <0.5 cm in infants (<1.5 cm in adults)
    2. Low malignant potential (especially pre-Puberty)
    3. Protocol
      1. Close observation by primary provider
      2. Refer lesions with change in color, texture, size, bleeding or pain
      3. If excised, it is safe to wait until after Puberty

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