Speckled Lentiginous Nevus 

  • Author: Rossitza Lazova, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 17, 2011
 

Background

Speckled lentiginous nevus is a patch of hyperpigmentation that can be seen on any area of the body. This patch contains a variable number of darkly pigmented macules and papules. Some authorities believe that speckled lentiginous nevus is a subtype of congenital melanocytic nevus.[1, 2]

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Pathophysiology

Speckled lentiginous nevus may represent a localized defect in neural crest melanoblasts that populate a particular area of the skin. Environmental and genetic factors may also play a role. Mosaicism may be an explanation for the development of zosteriform speckled lentiginous nevus.[3]

Speckled lentiginous nevus can be associated with different disorders. In facial features, anorexia, cachexia, and eye and skin anomalies (FACES) syndrome, cutaneous findings include zosteriform speckled lentiginous nevi. Associated findings also include ichthyosis, Ebstein syndrome (the eponym for congenital downward displacement of the tricuspid valve into the right ventricle), epidermal nevi, nevus sebaceous, scleral pigmentation, segmental neurofibromatosis type I,[4] adult-onset hearing loss, corneal snowflake dystrophy, and hypertrophy of the underlying pectoralis major muscle.

An association with phacomatosis pigmentovascularis and phacomatosis pigmentokeratotica has also been described.[5, 6, 7, 8, 9]

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Epidemiology

Frequency

United States

In the general population, the prevalence of speckled lentiginous nevi is similar to that of congenital melanocytic nevi, with a prevalence of 0.2-2.3%, depending on age.[10]

International

Speckled lentiginous nevus has been studied in Canadian children of Asian and white European origins, and its prevalence is the same in both ethnic groups.

Mortality/Morbidity

Speckled lentiginous nevus is a benign neoplasm. In rare instances of malignant transformation within a speckled lentiginous nevus, morbidity and mortality are dependent on the stage of the secondary malignancy. At least 20 cases of cutaneous melanoma developing within a speckled lentiginous nevus have been reported.[11, 12, 13, 14, 15]

Race

No racial predilection is noted.

Sex

No sexual predilection is recognized.

Age

  • Low prevalence rates of up to 0.2% have been reported in series of newborns. Approximately 80% of speckled lentiginous nevi appear at birth or during early infancy. They may present as lightly colored café au lait macules at birth, which later develop background hyperpigmentation and darkly pigmented macules and papules over months, years, or sometimes decades.
  • The prevalence rate of speckled lentiginous nevus is 1.3-2.1% in school-aged children and adolescents.
  • In adults, the frequency of speckled lentiginous nevi larger than 1.5 cm in diameter is 2.3%.
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Contributor Information and Disclosures
Author

Rossitza Lazova, MD  Associate Professor of Dermatology and Pathology, Director of Dermatopathology Residency and Fellowship Program, Yale University School of Medicine; Consulting Pathologist/Dermatopathologist, Veterans Affairs Medical Center, West Haven, Connecticut

Rossitza Lazova, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michelle Pelle, MD  Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California at San Diego

Michelle Pelle, MD is a member of the following medical societies: American Academy of Dermatology, California Medical Association, Medical Dermatology Society, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Christen M Mowad, MD  Associate Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgments

The author would like to thank Dr. Jean Bolognia for generously providing the clinical photos.

References

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  2. Torrelo A, de Prada I, Zambrano A, Happle R. Extensive speckled lentiginous nevus associated with giant congenital melanocytic nevus: an unusual example of twin spotting?. Eur J Dermatol. Nov-Dec 2003;13(6):534-6. [Medline].

  3. Altman DA, Banse L. Zosteriform speckled lentiginous nevus. J Am Acad Dermatol. Jul 1992;27(1):106-8. [Medline].

  4. Chen W, Fan PC, Happle R. Partial unilateral lentiginosis with ipsilateral Lisch nodules and axillary freckling. Dermatology. 2004;209(4):321-4. [Medline].

  5. Boente Mdel C, Asial RA, Happle R. Phacomatosis pigmentokeratotica: a follow-up report documenting additional cutaneous and extracutaneous anomalies. Pediatr Dermatol. Jan-Feb 2008;25(1):76-80. [Medline].

  6. Happle R. Dohi Memorial Lecture. New aspects of cutaneous mosaicism. J Dermatol. Nov 2002;29(11):681-92. [Medline].

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  8. Happle R. Speckled lentiginous nevus syndrome: delineation of a new distinct neurocutaneous phenotype. Eur J Dermatol. Mar-Apr 2002;12(2):133-5. [Medline].

  9. Jordaan HF, Happle R. Phacomatosis spilorosea associated with lymphoedema. Br J Dermatol. Aug 2008;159(2):489-91. [Medline].

  10. Kopf AW, Levine LJ, Rigel DS, Friedman RJ, Levenstein M. Prevalence of congenital-nevus-like nevi, nevi spili, and cafe au lait spots. Arch Dermatol. Jun 1985;121(6):766-9. [Medline].

  11. Borrego L, Hernandez Santana J, Baez O, Hernandez Hernandez B. Naevus spilus as a precursor of cutaneous melanoma: report of a case and literature review. Clin Exp Dermatol. Nov 1994;19(6):515-7. [Medline].

  12. Brufau C, Moran M, Armijo M. [Nevus on nevus. Apropos of 7 case reports, 3 of them associated with other dysplasias, and 1 with an invasive malignant melanoma]. Ann Dermatol Venereol. 1986;113(5):409-18. [Medline].

  13. Martinez-Menchon T, Mahiques Santos L, Vilata Corell JJ, Febrer Bosch I, Fortea Baixauli JM. Phacomatosis pigmentokeratotica: a 20-year follow-up with malignant degeneration of both nevus components. Pediatr Dermatol. Jan-Feb 2005;22(1):44-7. [Medline].

  14. Rhodes AR, Mihm MC Jr. Origin of cutaneous melanoma in a congenital dysplastic nevus spilus. Arch Dermatol. Apr 1990;126(4):500-5. [Medline].

  15. Vaidya DC, Schwartz RA, Janniger CK. Nevus spilus. Cutis. Dec 2007;80(6):465-8. [Medline].

  16. Casanova D, Bardot J, Aubert JP, Andrac L, Magalon G. Management of nevus spilus. Pediatr Dermatol. May-Jun 1996;13(3):233-8. [Medline].

  17. Bolognia JL. Speckled lentigenous nevus (nevus spilus). In: Nordlund JJ, Boissy RE, Hearing VJ, et al, eds. The Pigmentary System. London, England: Blackwell Science; 1998:958-66.

  18. Cohen HJ, Minkin W, Frank SB. Nevus spilus. Arch Dermatol. Oct 1970;102(4):433-7. [Medline].

  19. Haenssle HA, Kaune KM, Buhl T, Thoms KM, Padeken M, Mitteldorf C. Large speckled lentiginous naevus superimposed with Spitz naevi: sequential digital dermoscopy may lead to unnecessary excisions triggered by dynamic changes. Clin Exp Dermatol. Mar 2009;34(2):212-5. [Medline].

  20. Kurban RS, Preffer FI, Sober AJ, Mihm MC Jr, Barnhill RL. Occurrence of melanoma in "dysplastic" nevus spilus: report of case and analysis by flow cytometry. J Cutan Pathol. Oct 1992;19(5):423-8. [Medline].

  21. Stewart DM, Altman J, Mehregan AH. Speckled lentiginous nevus. Arch Dermatol. Jun 1978;114(6):895-6. [Medline].

  22. Torchia D. More on speckled lentiginous nevus syndrome. Dermatology. 2010;221(4):324-5. [Medline].

  23. Torti DC, Brennick JB, Storm CA, Dinulos JG. Spitz Nevi Arising in Speckled Lentiginous Nevus: Clinical, Histologic, and Molecular Evaluation of Two Cases. Pediatr Dermatol. Mar 3 2011;[Medline].

  24. Vente C, Neumann C, Bertsch H, Rupprecht R, Happle R. Speckled lentiginous nevus syndrome: report of a further case. Dermatology. 2004;209(3):228-9. [Medline].

  25. Vidaurri-de la Cruz H, Happle R. Two distinct types of speckled lentiginous nevi characterized by macular versus papular speckles. Dermatology. 2006;212(1):53-8. [Medline].

 
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A large unilateral speckled lentiginous nevus that contains numerous small dark macules.
Extensive speckled lentiginous nevus.
Prominent basal layer pigmentation similar to that seen in lentigo simplex (hematoxylin-eosin stain, original magnification X200).
Elongated rete ridges and lentiginous proliferation of melanocytes at the dermal-epidermal junction (hematoxylin-eosin stain, original magnification X200).
Junctional melanocytic nevus composed of small nests of melanocytes and lentiginous melanocytic proliferation at the dermal-epidermal junction (hematoxylin-eosin stain, original magnification X100).
Compound melanocytic nevus with nests of uniform melanocytes in the epidermis and the dermis (hematoxylin-eosin stain, original magnification X200).
 
 
 
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