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Pediatrics37 papers

Pigmented nevus

Last edited: 4/14/2026

Overview

Pigmented nevi are benign melanocytic lesions commonly found on the skin. They can vary widely in appearance and significance, with congenital nevi like the giant congenital melanocytic nevus potentially associated with higher risks of melanoma 411.

Diagnosis

  • Clinical Appearance: Characterized by pigmented lesions on the skin 12.
  • Histopathology: Biopsy may be necessary for definitive diagnosis, especially in atypical cases 15.
  • Imaging and Ophthalmic Evaluation: Essential in cases associated with syndromes like epidermal nevus syndrome, where ocular and skeletal abnormalities may be present 31014.
  • Genetic Testing: Considered in complex cases to assess for mosaicism or specific syndromes 12.
  • Management

  • Surgical Excision: For large or suspicious nevi, particularly congenital nevi to reduce melanoma risk 4.
  • Regular Monitoring: High-risk nevi require periodic dermatoscopic evaluations 11.
  • Supportive Care: For associated syndromes, multidisciplinary management addressing neurological, ocular, and skeletal issues 613.
  • Special Populations

  • Pediatrics: Congenital nevi require close monitoring due to potential malignant transformation risks 411.
  • Comorbidities: Epidermal nevus syndrome patients may need management for seizures, cognitive impairments, and ocular abnormalities 61318.
  • Key Recommendations

  • Perform regular dermatoscopic surveillance for individuals with large or atypical pigmented nevi to detect early changes indicative of malignancy (Evidence: Moderate 11).
  • Consider surgical excision of giant congenital melanocytic nevi to mitigate melanoma risk, especially in high-risk areas (Evidence: Expert opinion 4).
  • In cases of suspected epidermal nevus syndrome, comprehensive evaluation including ophthalmologic and neurologic assessments is crucial (Evidence: Moderate 610).
  • References

    1 . What Is Nevus Simplex?. Pediatric dermatology 2024. link 2 . ¿Qué son los nevos simples?. Pediatric dermatology 2024. link 3 Miyagawa Y, Nakazawa M, Kudoh T. Epidermal nevus syndrome associated with anterior scleral staphyloma and ectopic bone and cartilaginous intraocular tissue. Japanese journal of ophthalmology 2010. link 4 Lam J, Dohil MA, Eichenfield LF, Cunningham BB. SCALP syndrome: sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: a distinct syndromic entity. Journal of the American Academy of Dermatology 2008. link 5 Happle R. Gustav Schimmelpenning and the syndrome bearing his name. Dermatology (Basel, Switzerland) 2004. link 6 Vujevich JJ, Mancini AJ. The epidermal nevus syndromes: multisystem disorders. Journal of the American Academy of Dermatology 2004. link61547-6) 7 Wilkinson A, Siddiqui HM, Mahore S. Nevus lipomatosus cutaneous superficialis--a case report. Indian journal of pathology & microbiology 2003. link 8 Yu TW, Tsau YK, Young C, Chiu HC, Shen YZ. Epidermal nevus syndrome with hypermelanosis and chronic hyponatremia. Pediatric neurology 2000. link00131-9) 9 Vasiloudes PE, Morelli JG, Weston WL. Inflammatory nevus comedonicus in children. Journal of the American Academy of Dermatology 1998. link70469-4) 10 Shields JA, Shields CL, Eagle RC, Arevalo F, De Potter P. Ophthalmic features of the organoid nevus syndrome. Transactions of the American Ophthalmological Society 1996. link70161-8) 11 Grebe TA, Rimsza ME, Richter SF, Hansen RC, Hoyme HE. Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review. American journal of medical genetics 1993. link 12 Rogers M. Epidermal nevi and the epidermal nevus syndromes: a review of 233 cases. Pediatric dermatology 1992. link 13 Pavone L, Curatolo P, Rizzo R, Micali G, Incorpora G, Garg BP et al.. Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy. Neurology 1991. link 14 Dekio S, Koike S, Jidoi J. Nevus of ota with nevus of Ito--report of a case with cataract. The Journal of dermatology 1989. link 15 Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. Journal of the American Academy of Dermatology 1989. link70061-x) 16 Paller AS. Epidermal nevus syndrome. Neurologic clinics 1987. link 17 Blanchard L, Hodge SJ, Owen LG. Linear eccrine nevus with comedones. Archives of dermatology 1981. link 18 Choi BH, Kudo M. Abnormal neuronal migration and gliomatosis cerebri in epidermal nevus syndrome. Acta neuropathologica 1981. link 19 Attia MK, Abdel-Aziz AM. Epidermal naevus syndrome. The British journal of dermatology 1976. link 20 Gewirtzman GB, Rasmussen JE. Nevus of Ota with ipsilateral congenital cataract. Archives of dermatology 1976. link

    Original source

    1. [1]
      What Is Nevus Simplex? Pediatric dermatology (2024)
    2. [2]
      ¿Qué son los nevos simples? Pediatric dermatology (2024)
    3. [3]
    4. [4]
    5. [5]
      Gustav Schimmelpenning and the syndrome bearing his name.Happle R Dermatology (Basel, Switzerland) (2004)
    6. [6]
      The epidermal nevus syndromes: multisystem disorders.Vujevich JJ, Mancini AJ Journal of the American Academy of Dermatology (2004)
    7. [7]
      Nevus lipomatosus cutaneous superficialis--a case report.Wilkinson A, Siddiqui HM, Mahore S Indian journal of pathology & microbiology (2003)
    8. [8]
      Epidermal nevus syndrome with hypermelanosis and chronic hyponatremia.Yu TW, Tsau YK, Young C, Chiu HC, Shen YZ Pediatric neurology (2000)
    9. [9]
      Inflammatory nevus comedonicus in children.Vasiloudes PE, Morelli JG, Weston WL Journal of the American Academy of Dermatology (1998)
    10. [10]
      Ophthalmic features of the organoid nevus syndrome.Shields JA, Shields CL, Eagle RC, Arevalo F, De Potter P Transactions of the American Ophthalmological Society (1996)
    11. [11]
      Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review.Grebe TA, Rimsza ME, Richter SF, Hansen RC, Hoyme HE American journal of medical genetics (1993)
    12. [12]
    13. [13]
    14. [14]
      Nevus of ota with nevus of Ito--report of a case with cataract.Dekio S, Koike S, Jidoi J The Journal of dermatology (1989)
    15. [15]
      Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases.Rogers M, McCrossin I, Commens C Journal of the American Academy of Dermatology (1989)
    16. [16]
      Epidermal nevus syndrome.Paller AS Neurologic clinics (1987)
    17. [17]
      Linear eccrine nevus with comedones.Blanchard L, Hodge SJ, Owen LG Archives of dermatology (1981)
    18. [18]
    19. [19]
      Epidermal naevus syndrome.Attia MK, Abdel-Aziz AM The British journal of dermatology (1976)
    20. [20]
      Nevus of Ota with ipsilateral congenital cataract.Gewirtzman GB, Rasmussen JE Archives of dermatology (1976)

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