eMedicine Specialties > Dermatology > Benign Neoplasms

Speckled Lentiginous Nevus

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
Contributor Information and Disclosures

Updated: Mar 23, 2009

Introduction

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

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

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%.

Clinical

History

  • Patients may complain of hyperpigmented or multicolored skin lesions, with colors most often including shades of tan, brown, and black.
  • The lesions are present at birth or noted during the individual's first years of life.
  • Patients may be aware of an increase in the size of the lesions or changes in color over time.
  • Skin distribution varies, with the trunk being the most common site of occurrence.

Physical

  • A variable number of black, brown, or red-brown macules and papules are seen within a patch of tan-to-brown hyperpigmentation.
  • The background pigmented macule or patch is usually 3-6 cm in diameter, but the diameter may vary from 1 cm to more than 60 cm.
A large unilateral speckled lentiginous nevus tha...

A large unilateral speckled lentiginous nevus that contains numerous small dark macules.

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A large unilateral speckled lentiginous nevus tha...

A large unilateral speckled lentiginous nevus that contains numerous small dark macules.

  • The lesions are usually oval, but they can be linear. Some follow the lines of Blaschko or have a sharp demarcation at the midline. An extensive amount of body surface area may be involved.
Extensive speckled lentiginous nevus.

Extensive speckled lentiginous nevus.

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Extensive speckled lentiginous nevus.

Extensive speckled lentiginous nevus.

  • The macules (speckles) and papules lying within the background patch range from lentigines to junctional, compound, or dermal nevi.
    • Spitz nevi and blue nevi may also occur.
    • The speckles range from 1-9 mm; most commonly, they are 2-3 mm in diameter.
    • To the author's knowledge, deep penetrating nevus occurring within a speckled lentiginous nevus has not been reported.

Causes

  • Speckled lentiginous nevi may represent a defect in the melanoblasts that populate a localized area of the skin.
  • Genetic and environmental factors may also play a role in their occurrence.

More on Speckled Lentiginous Nevus

Overview: Speckled Lentiginous Nevus
Differential Diagnoses & Workup: Speckled Lentiginous Nevus
Treatment & Medication: Speckled Lentiginous Nevus
Follow-up: Speckled Lentiginous Nevus
Multimedia: Speckled Lentiginous Nevus
References
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References

  1. Schaffer JV, Orlow SJ, Lazova R, Bolognia JL. Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol. Feb 2001;137(2):172-8. [Medline].

  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].

  7. Happle R. Speckled lentiginous naevus: which of the two disorders do you mean?. Clin Exp Dermatol. Mar 2009;34(2):133-5. [Medline].

  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. 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].

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

  21. 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].

  22. 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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Further Reading

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Keywords

speckled lentiginous nevus, speckled lentiginous nevi, nevus spilus, zosteriform speckled lentiginous nevus, speckled zosteriform lentiginous nevus, mosaic speckled lentiginous nevus, nevus on nevus, naevus sur naevus, nevus sobre nevus, speckled nevus spilus, nevus spilus zoniforme, nevus spilus en nappe, spotty nevus, congenital melanocytic nevus

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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.

Medical Editor

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.

Pharmacy Editor

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
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.

Managing Editor

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.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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