What is the recommended approach for managing an intradermal nevus (mole) with congenital features?

Management of Intradermal Nevus with Congenital Features

For intradermal nevi with congenital features, referral to dermatology is recommended for thorough evaluation and management, with complete excision considered for concerning lesions. 1, 2

Risk Assessment and Classification

• Congenital melanocytic nevi (CMN) occur in approximately 1-3.6% of newborns and carry a lifetime melanoma risk of 0.7-1.7% 2
• CMN are classified into three main subtypes: "Classic" (brown/black macules, papules, patches or plaques), Blue (gray-blue patches or plaques), and Nevus spilus (brown patch with darker overlying macules/papules) 1, 2
• Risk factors for melanoma development include:
  • Size >40 cm in projected adult diameter 1
  • Multiple "satellite" nevi 1
  • Trunk location 1
  • Presence of numerous CMN 1

Diagnostic Approach

• Initial evaluation should include:
  • Visual inspection (with dermoscopic evaluation when available) 2
  • Thorough palpation of the nevus 1
  • Regional lymph node examination 1
  • Serial photographs to monitor changes over time 1, 2
• Palpation is particularly important as melanoma in CMN can present as deep nodules without overlying color change 1
• For intradermal nevi with congenital features, careful assessment for concerning changes is essential as rare cases of melanoma arising from intradermal nevi have been reported 3

Management Protocol

• Referral to dermatology is recommended for:
  • Large, giant, or multiple CMN of any size 1, 2
  • Small/medium CMN with concerning features (color variation, nodules, symptoms) 1
  • Intradermal nevi with congenital features showing any suspicious changes 2
• Follow-up frequency should be determined by:
  • Size and characteristics of the nevus 1
  • Patient's age 1
  • Presence of concerning features 1
• For larger, multiple, or changing nevi, follow closely during infancy and puberty (times of expected nevus change) with visits every 3 months initially 1
• After the first year of life, in the absence of concerns, visit frequency can gradually decrease to yearly dermatologist evaluations 1

Monitoring and Surveillance

• Between visits, patients/caregivers should:
  • Visually inspect and palpate the nevus regularly 1
  • Report concerning changes immediately (rapid growth, bleeding, pain, nodules, ulceration) 1, 2
• Normal changes that may occur in intradermal nevi with congenital features include:
  • Spontaneous lightening (especially in scalp lesions) 1
  • Changes in pigmentation pattern (mottled or speckled) 1
  • Textural changes (becoming more raised, verrucous, or papillated) 1

Surgical Considerations

• Complete excision should be considered when:
  • Suspicious changes are noted 1, 3
  • The lesion is in a location amenable to surgery 1
  • The patient's age and health status permit 1
• The decision for procedural intervention or removal is complex and requires detailed risk-benefit discussions considering:
  • Family preference 1
  • Size and location of the nevus 1
  • Patient age and overall health 1
• While some older literature advocated for prophylactic excision of all congenital nevi 4, current guidelines take a more nuanced approach based on risk assessment 1, 2

Skin Care Recommendations

• Bathing with water alone or with a nonsoap cleanser 2-3 times weekly followed by application of bland emollient 1
• For pruritus or eczematous changes:
  • Use bland, thick emollients (minimal fragrances/preservatives) for chronic management 1
  • Add low to mid-potency topical corticosteroids twice daily for acute flares 1
• Follow standard photoprotection recommendations (sunscreen, hats, protective clothing, seeking shade) 1
• For ulcerations or erosions, cleanse with soap/nonsoap cleanser and water, then apply petroleum jelly or bland ointment with a bandage 1

Important Caveats

• Flat melanocytic areas adjacent to intradermal nevi should raise suspicion and may warrant excision with histopathological evaluation 3
• Histopathologic examination of concerning changes should be performed by a dermatopathologist with expertise in pediatric pigmented lesions 2
• While the risk of malignant transformation in intradermal nevi is low, vigilant monitoring is still recommended, particularly in older patients 3, 5