congenital melanocytic nevus
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Introduction
May be premalignant lesions, especially giant melanocytic nevi.
Etiology
- developmental defect in neural crest-derived melanoblasts
- defect problably occurs after 10 weeks in utero, but before 6th uterine month
Epidemiology
- present at birth
- some lesions may become visible after birth "fading in" as a large lesion over a period of weeks (tardive)
- equal prevalence in males & females
- all races
- 1% of white newborns - most < 3 cm in diameter
Clinical manifestations
- present at birth
- isolated lesions, may be found on any skin surface
- variably sized, may cover entire body parts
- medium-dark brown, variegated color across lesion
- generally round or oval with regular or irregular borders
- generally a plaque with rough or lobulated surface, increasingly so with time
- coarse hair growth
- may involve leptomeninges if large lesion occurs on head
- seizures
- focal neurologic deficits
- obstructive hydrocephalus
Complications
- large or giant congenital melanocytic nevus
- risk of melanoma is 6.3%
- diagnosis generally made between 3-5 years of age
- prognosis is poor
- small lesions - lifetime risk of melanoma is 1-5%
Differential diagnosis
- common acquired melanocytic nevus
- dysplastic melanocytic nevus
- congenital blue nevus
- nevus spilus
- Becker's nevus
- pigmented epidermal nevus
- cafe-au-lait macules
Management
- small nevi not known to be present at birth should be assumed to be acquired & managed according to appearance
- atypical appearing lesions should be surgically excised
- small congenital melanocytic nevi should be removed before age 12
- large nevi > 1.5 cm
- periodic follow-up for life
- prophylactic surgical excision
- excision with full-thickness skin graft as indicated
- swing flaps, tissue expanders for large lesions
- giant lesions
- risk of developing melanoma is significant before age 3-5
- lesion should be excised as soon as possible
- techiniques using patients own skin grown in tissue culture
- tissue expanders