Skin melanocytic tumor
Nevus with features of congenital onset

Topic Completed: 1 November 2012

Minor changes: 15 January 2021

Copyright: 2003-2021,, Inc.

PubMed Search: Congenital nevus [title]

Related topics: Giant congenital nevus

Christopher S. Hale, M.D.
Page views in 2020: 5,878
Page views in 2021 to date: 1,181
Cite this page: Hale CS. Nevus with features of congenital onset. website. Accessed March 3rd, 2021.
Definition / general
  • Most rigidly definition is nevi present at birth
  • Disagreement regarding whether nevi presenting very early in life but not at birth can be considered congenital (Acta Derm Venereol 2012;92:586)
  • In practice, nevi often labeled congenital if they have "congenital features" of melanocytes clustered around follicles, adnexae, nerves and between collagen fibers at base of lesion
  • References: eMedicine, J Am Acad Dermatol 2012;67:495.e1
  • Agminate / agminated nevus: nevi that are "clustered", and may be confined to a developmental segment (J Invest Dermatol 2011;131:788)
  • Nevus spilus / speckled lentiginous nevus: common type of agminate nevus with multiple pigmented macules or papules within a pigmented patch (Cutis 2007;80:465)
  • Neuronevus: compound nevi with prominent neural features, Masson's neuronevus (cellular blue nevus with neural / schwannian differentiation) or congenital nevus with prominent neural features
  • Giant congenital nevus: see below
Clinical features
Case reports
Clinical images

Contributed by Mark R. Wick, M.D.
Missing Image

Breast skin

Images hosted on other servers:
Missing Image

Reticular pattern and regular globules

Missing Image

Globular pattern and patchy network

Missing Image

Haloed globules (3)

Missing Image Missing Image

Target network and target globules (4 and 5)

Gross images

Case #383

Partial black discoloration

Microscopic (histologic) description
  • Tends to involve reticular dermis, subcutis, skin adnexa, arrector pili muscles and nerves with single cell permeation of collagen
  • Also neural differentiation with Wagner-Meissner-like corpuscles
  • Frequent proliferative nodules
  • Lesions of infants may have pagetoid melanocytic proliferation
  • Proliferative nodules:
    • Dermal nodules of large epithelioid or spindled melanocytes that merge with surrounding nevus cells
    • Often prominent nucleoli, cellular areas, focal hemorrhage and ulceration but no necrosis, no destructive growth, minimal inflammation and 0 - 4 mitotic figures/10 HPF
    • Lesions mature and regress over time
Microscopic (histologic) images

Case #383

Various images

Images hosted on other servers:
Missing Image Missing Image

Symmetrical broad proliferation of melanocytes
in papillary and reticular dermis with maturation,
splaying between collagen bundles, permeation of
muscles of hair erection, blood vessels, adnexa

Missing Image

Deep congenital nevus

Molecular / cytogenetics description
  • Lesions present at birth usually have NRAS but not BRAF mutations
  • Lesions with congenital type histologic features but not present at birth more commonly have BRAF but not NRAS mutations (J Invest Dermatol 2007;127:179)
  • Germline Melanocortin-1-Receptor (MC1R) genotype is associated with severity of cutaneous phenotype in congenital melanocytic nevi (J Invest Dermatol 2012;132:2026)
Differential diagnosis
Back to top
Image 01 Image 02