Dermatology Book


Congenital Melanocytic Nevus

Aka: Congenital Melanocytic Nevus, Congenital Melanocytic Nevi, Garment Nevi, Giant Congenital Nevi, Birthmark, Giant Hairy Nevi, Bathing Trunk Nevi, Congenital Nevus, Congenital Nevi
  1. See Also
    1. Atypical Nevus
    2. Melanoma
    3. Melanoma Risk Factors
    4. Speckled Lentiginous Nevus
  2. Epidemiology
    1. Prevalence: Up to 2% of newborns
  3. Pathophysiology
    1. Neural crest Melanocytes and their precursors with disrupted migration
  4. Signs
    1. Brown to black lesions that are typically flat (but can be elevated, and may thicken as children get older)
    2. Size can vary from a few millimeters to several centimeters to involving an entire region of the body
    3. Hair is variably present
    4. Typically with uniform pigmentation
  5. 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
  6. Lab: Histology
    1. Localized to the lower Dermis
  7. Differential Diagnosis
    1. Cafe-Au-Lait Spots
  8. 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
  9. Management
    1. 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)
        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)
    2. 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
    3. 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. If excised, it is safe to wait until after Puberty
  10. References
    1. Habif (2003) Clinical Dermatology, 4th ed.. Mosby, p. 773-813
    2. Berg (2003) Melanoma Res 13:441-5 [PubMed]
    3. Krengel (2006) Br J Dermatol 155:1-8 [PubMed]
    4. McLaughlin (2008) Am Fam Physician 77:56-60 [PubMed]

Birthmark (C0265974)

Definition (MEDLINEPLUS)

Birthmarks are abnormalities of the skin that are present when a baby is born. There are two types of birthmarks. Vascular birthmarks are made up of blood vessels that haven't formed correctly. They are usually red. Two types of vascular birthmarks are hemangiomas and port-wine stains. Pigmented birthmarks are made of a cluster of pigment cells which cause color in skin. They can be many different colors, from tan to brown, gray to black, or even blue. Moles can be birthmarks.

No one knows what causes many types of birthmarks, but some run in families. Most birthmarks are not serious, and some go away on their own. Your baby's doctor will look at the birthmark to see if it needs any treatment or if it should be watched. Pigmented birthmarks aren't usually treated, except for moles. Treatment for vascular birthmarks includes laser surgery.

Concepts Congenital Abnormality (T019)
ICD10 Q82.5
SnomedCT 51089004, 268354001, 205561006
English Birth mark, unspecified, Birthmark of skin, Birthmark NOS, birthmarks, birthmark disorders, birthmark, Birthmarks, Birth mark, unspecified (disorder), Birthmark, Birthmark (disorder), Birthmark of skin, NOS, Birthmark, NOS
Spanish marca de nacimiento, no especificada (trastorno), marca de nacimiento, no especificada, mancha de nacimiento de la piel (trastorno), mancha de nacimiento de la piel, mancha de nacimiento
Derived from the NIH UMLS (Unified Medical Language System)

Congenital melanocytic nevus (C1318558)

Definition (NCI) A melanocytic nevus that is present at birth. It may present as a small macular, papular, or plaque-like lesion or as a large brown to black hairy skin lesion.
Concepts Neoplastic Process (T191)
SnomedCT 254203006, 84953004, 400151006, 398696001, 398943008
English Congen melanocytic naevus skin, Congen melanocytic nevus skin, Congen pigmented naevus skin, Congen pigmented nevus skin, Congenital Nevus of the Skin, Congenital Nevus of Skin, Congenital Skin Nevus, Congenital Melanocytic Nevus, congenital pigmented naevus, congenital melanocytic naevus, congenital melanocytic nevus, nevus pigmented congenital, congenital pigmented nevus, Congenital melanocytic nevus of skin (disorder), Congenital melanocytic nevus (disorder), Congenital Melanocytic Nevi, Congenital Pigmented Melanocytic Nevus, Congenital melanocytic naevus, Congenital melanocytic naevus of skin, Congenital melanocytic nevus of skin, Congenital pigmented naevus, Congenital pigmented naevus of skin, Congenital pigmented nevus, Congenital pigmented nevus of skin, Congenital dermal melanocytic naevus, Congenital dermal melanocytic nevus, Congenital melanocytic nevus (morphologic abnormality), Congenital pigmented melanocytic naevus of skin, Congenital pigmented melanocytic naevus, Congenital pigmented melanocytic nevus (disorder), Congenital pigmented melanocytic nevus of skin (disorder), Congenital pigmented melanocytic nevus of skin, Congenital pigmented melanocytic nevus, Congenital melanocytic nevus, Congenital melanocytic nevus, NOS, Congenital Melanocytic Nevus of Skin, Congenital Melanocytic Nevus of the Skin, Congenital Pigmented Nevus of Skin, Congenital Pigmented Nevus of the Skin, Congenital Pigmented Skin Nevus
Czech Vrozený melanocytový névus, Vrozený melanocytární névus
Dutch congenitale melanocytische naevus
French Naevus mélanocytaire congénital
German kongenitaler melanozytaerer Naevus
Hungarian Veleszületett melanocytás naevus
Italian Nevo melanocitico congenito
Japanese 先天性メラノサイト性母斑, センテンセイメラノサイトセイボハン
Portuguese Nevo melanocítico congénito
Spanish Nevus melanocítico congénito, nevo melanocítico congénito (trastorno), nevo congénito melanocítico pigmentado (trastorno), nevo congénito melanocítico pigmentado, nevo congénito pigmentado melanocítico de piel, nevo melanocítico congénito (anomalía morfológica), nevo melanocítico congénito, nevus congénito pigmentado melanocítico de piel (trastorno), nevus congénito pigmentado melanocítico de piel, nevus melanocítico congénito
Derived from the NIH UMLS (Unified Medical Language System)

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