Journal Mobile Options
Table of Contents
Vol. 212, No. 1, 2006
Issue release date: November 2005
Dermatology 2006;212:53–58

Two Distinct Types of Speckled Lentiginous Nevi Characterized by Macular versus Papular Speckles

Vidaurri-de la Cruz H. · Happle R.
Department of Dermatology, Philipp University of Marburg, Marburg, Germany

Individual Users: Register with Karger Login Information

Please create your User ID & Password

Contact Information

I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in


Background: Speckled lentiginous nevus (SLN; synonym: nevus spilus) is a darkly spotted light-brown macule that mostly occurs as an isolated lesion of rather limited dimensions but sometimes may involve large areas of the body. So far, this skin disorder has been considered to represent one clinical entity. Objective: Because SLN is occasionally associated with complex birth defects such as phacomatosis pigmentovascularis, phacomatosis pigmentokeratotica, or SLN syndrome, we tested our idea that two different types of SLN may exist, each one associated with a particular syndrome. Methods: A review of case reports on SLN published during the years 1970–2004 was performed. Results: This evaluation of cases provided evidence that two different types of SLN exist, in the form of macular versus papular SLN, each one being related to a specific syndrome. Macular SLN is characterized by a tannish-brown background with darker flat speckles. The distribution of speckles is rather even and resembles a polka-dot pattern. Histopathologically, this type of SLN is characterized by what has been called a ‘jentigo’ pattern in the darker speckles and by some nests of melanocytes at the dermoepidermal junction at the tips of the papillae, whereas the background pigmentation shows the microscopical features of a lentigo. Papular SLN is characterized by a light-brown macule superimposed by multiple melanocytic nevi in the form of papules or nodules that show a more uneven distribution reminiscent of a star map. Small dark macules may likewise be present. Histopathologically, the papular component consists of dermal or compound melanocytic nevi. A separation of the two types of SLN is important because our analysis showed that macular SLN is a hallmark of a particular type of phacomatosis pigmentovascularis, whereas papular SLN is typically present in phacomatosis pigmentokeratotica as well as in SLN syndrome. Conclusions: Macular SLN and papular SLN appear to be two distinct cutaneous entities. This dichotomy may turn out to be important when such nevi will be analyzed at the molecular level.

Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.


  1. Brufau C, Moran M, Armijo M: Naevus sur naevus, à propos de 7 observations, trois associées à d’autres dysplasies, et une à un mélanome malin invasif. Ann Dermatol Venereol 1986;113:409–418.
  2. Konrad K, Hönigsmann H, Wolff K: Naevus spilus – ein Pigmentnaevus mit Riesenmelanosomen: Klinik, Histologie und Ultrastruktur. Hautarzt 1974;25:585–593.
  3. Cohen HJ, Minkin W, Frank SB: Nevus spilus. Arch Dermatol 1970;102:433–437.
  4. Matsudo H, Reed WB, Homme D, Bartok W, Horowitz R: Zosteriform lentiginous nevus. Arch Dermatol 1973;107:902–905.
  5. Stewart DM, Altman J, Mehregan AH: Speckled lentiginous nevus. Arch Dermatol 1978;114:895–896.
  6. Schaffer JV, Orlow SJ, Lazova R, Bolognia JL: Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol 2001;137:172–178.
  7. Cohen LM: Nevus spilus: congenital or acquired ? Arch Dermatol 2001;137:215–216.
  8. Vion B, Belaïch S, Grossin M, Préaux J: Les aspects évolutifs du naevus sur naevus: revue de la littérature à propos de 7 observations. Ann Dermatol Venereol 1985;112:813–819.
  9. McLean DI, Gallagher RP: ‘Sunburn’ freckles, café-au-lait macules, and other pigmented lesions of schoolchildren: the Vancouver Moles Study. J Am Acad Dermatol 1995;32:565–570.
  10. Sigg C, Pelloni F, Schnyder UW: Frequency of congenital nevi, nevi spili and café-au-lait spots and their relation to nevus count and skin complexion in 939 children. Dermatologica 1990;180:118–123.
  11. Kopf AW, Levine LJ, Rigel DS, Friedman RJ, Levenstein M: Prevalence of congenital-nevus-like nevi, nevi spili, and café au lait spots. Arch Dermatol 1985;121:766–769.
  12. Crosti C, Betti R: Inherited extensive speckled lentiginous nevus with ichthyosis: report of a previously undescribed association. Arch Dermatol 1994;130:393–395.
  13. Roma P, Sanjeev H, Bhusan K: Generalized naevus spilus: a rare entity? J Eur Acad Dermatol Venereol 2000;14:430.
  14. Betti R, Inselvini E, Crosti C: Extensive unilateral speckled lentiginous nevus. Am J Dermatopathol 1994;16:554–556.
  15. Stern JB, Haupt HM, Aaronson CM: Malignant melanoma in a speckled zosteriform lentiginous nevus. Int J Dermatol 1990;29:583–584.
  16. Bolognia JL: Fatal melanoma arising in a zosteriform speckled lentiginous nevus. Arch Dermatol 1991;127:1240–1241.
  17. Altman DA, Banse L: Zosteriform speckled lentiginous nevus. J Am Acad Dermatol 1992;27:106–108.
  18. Horio T, Ogawa M: Pigmentovascular nevus. Arch Dermatol 1973;107:463–464.
  19. Mahroughan M, Mehregan AH, Mehregan DA: Phakomatosis pigmentovascularis: report of a case. Pediatr Dermatol 1996;13:36–38.
  20. Prigent F, Kahn A, Martinet C, Saigot T: Phacomatose pigmento-vasculaire IIIa. Ann Dermatol Venereol 1991;118:531–533.
  21. Sigg C, Pelloni F: Oligosymptomatic form of Klippel-Trenaunay-Weber syndrome associated with giant nevus spilus. Arch Dermatol 1989;125:1284–1285.
  22. Zahorcsek Z, Schmelás A, Schneider I: Progrediente zirkumskripte Lentiginose – Phakomatosis pigmentovascularis III/A. Hautarzt 1988;39:519–523.
  23. Libow LF: Phakomatosis pigmentovascularis type IIIb. J Am Acad Dermatol 1993;29:305–307.
  24. Guiglia MC, Prendiville JS: Multiple granular cell tumors associated with giant speckled lentiginous nevus and nevus flammeus in a child. J Am Acad Dermatol 1991;24:359–363.
  25. Bielsa I, Paradelo C, Ribera M, Ferrrándiz C: Generalized nevus spilus and nevus anemicus in a patient with a primary lymphedema: a new type of phakomatosis pigmentovascularis? Pediatr Dermatol 1998;15:293–295.
  26. Happle R, Hoffmann R, Restano L, Caputo R, Tadini G: Phacomatosis pigmentokeratotica: a melanocytic-epidermal twin nevus syndrome. Am J Med Genet 1996;65:363–365.
  27. Hermes B, Cremer B, Happle R, Henz BM: Phacomatosis pigmentokeratotica: a patient with the rare melanocytic-epidermal twin nevus syndrome. Dermatology 1997;194:77–79.
  28. Tadini G, Ermacora E, Carminati G, Gelmetti C, Cambiaghi S, Brusasco A, Caputo R, Happle R: Unilateral speckled-lentiginous naevus, contralateral verrucous epidermal naevus, and diffuse ichthyosis-like hyperkeratosis: an unusual example of twin spotting? Eur J Dermatol 1995;5:659–663.

    External Resources

  29. Tadini G, Restano L, González-Pérez R, González-Enseñat A, Vincente-Villa MA, Cambiaghi S, Marchettini P, Mastrangelo M, Happle R: Phacomatosis pigmentokeratotica: report of new cases and further delineation of the syndrome. Arch Dermatol 1998;134:333–337.
  30. Goldberg LH, Collins SAB, Siegel DM: The epidermal nevus syndrome: case report and review. Pediatr Dermatol 1987;4:27–33.
  31. Boente MC, Pizzi de Parra N, Larralde-de Luna M, Bibas-Bonet H, Santos-Muñoz A, Parra V, Gramajo P, Moreno S, Asial RA: Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting. Eur J Dermatol 2000;10:190–194.
  32. Wauschkuhn J, Rohde B: Systematisierte Talgdrüsen-, Pigment- und epitheliale Naevi mit neurologischer Symptomatik: Feuerstein-Mimssches neuroektodermales Syndrom. Hautarzt 1971;22:10–13.
  33. Happle R: Speckled lentiginous nevus syndrome: delineation of a new distinct neurocutaneous phenotype. Eur J Dermatol 2002;12:133–135.
  34. Marchesi L, Naldi L, Di Landro A, Cavalieri d’Oro L, Brevi A, Cainelli T: Segmental lentiginosis with ‘jentigo’ histologic pattern. Am J Dermatopathol 1992;14:323–327.
  35. Sato S, Kato H, Hidano A: Divided nevus spilus and divided form of spotted grouped pigmented nevus. J Cutan Pathol 1979;6:507–512.
  36. Rütten A, Goos M: Nevus spilus with malignant melanoma in a patient with neurofibromatosis. Arch Dermatol 1990;126:539–540.
  37. Happle R: Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol 2005;141:385–388.
  38. Breitkopf C, Ernst K, Hundeiker M: Neubildungen auf Naevus spilus. Hautarzt 1996;47:759–762.
  39. Cox NH, Malcolm A, Long ED: Superficial spreading melanoma and blue naevus within naevus spilus – Ultrastructural assessment of giant pigment granules. Dermatology 1997;194:213–216.
  40. Krähn G, Thoma E, Peter RU: Zwei superfiziell spreitende maligne Melanome auf Naevus spilus. Hautarzt 1992;43:32–34.
  41. Wagner RF, Cottel WI: In situ malignant melanoma arising in a speckled lentiginous nevus. J Am Acad Dermatol 1989;20:125–126.
  42. Van der Horst JC, Dirksen HJ: Zosteriform lentiginous naevus. Br J Dermatol 1981;104:104–105.
  43. Page EV, Windhorst DB: Unusual nevus (nevus spilus). Arch Dermatol 1972;105:115.

    External Resources

  44. Ceylan C, Özdemir F, Öztürk G, Akalın T, Kılınç I: Tuberous sclerosis associated with multiple speckled lentiginous nevi. J Eur Acad Dermatol Venereol 2003;17:616–617.
  45. 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 2003;13:534–536.
  46. Mang R, Ruzicka T, Megahed M: Ungewöhnliche klinische Präsentationen melanozytärer Nävi. Hautarzt 2003;54:370–372.
  47. Kaur T, Kanwar AJ: Giant nevus spilus and centrofacial lentiginosis. Pediatr Dermatol 2004;21:516–517.
  48. Aloi F, Tomasini C, Pippione M: Agminated Spitz nevi occurring within a congenital speckled lentiginous nevus. Am J Dermatopathol 1995;17:594–598.
  49. Falo LD, Sober AJ, Barnhill RL: Evolution of a naevus spilus. Dermatology 1994;189:382–383.
  50. Misago N, Narisawa Y, Nishi T, Kohda H: Association of nevus sebaceus with an unusual type of ‘combined nevus’. J Cutan Pathol 1994;21:76–81.
  51. Kopf AW, Bart RS: Tumor conference #27: combined organoid and melanocytic nevus. J Dermatol Surg Oncol 1980;6:28–30.
  52. Betti R, Inselvini E, Palvarini M, Crosti C: Agminated intradermal Spitz nevi arising on an unusual speckled lentiginous nevus with localized lentiginosis: a continuum? Am J Dermatopathol 1997;19:524–527.
  53. Goldblum JR, Headington JT: Hypophosphatemic vitamin D-resistant rickets and multiple spindle and epithelioid nevi associated with linear nevus sebaceus syndrome. J Am Acad Dermatol 1993;29:109–111.
  54. Marulli GC, Campione E, Di Stefani A, Citarella L, Chimenti S: Ink spot lentigo arising on naevus spilus simulating melanoma. Acta Derm Venereol 2004;84:166–167.
  55. Langenbach N, Hohenleutner U, Landthaler M: Phacomatosis pigmentokeratotica: speckled lentiginous nevus in association with nevus sebaceus. Dermatology 1998;197:377–380.
  56. Hill VA, Felix RH, Mortimer PS, Harper JI: Phacomatosis pigmentokeratotica. J R Soc Med 2003;96:30–31.
  57. Asad S, Moss C: Evolving lesions of phakomatosis pigmentokeratotica. J Am Acad Dermatol 2004;50:P131.

    External Resources

  58. Vente C, Neumann C, Bertsch H, Rupprecht R, Happle R: Speckled lentiginous nevus syndrome: report of a further case. Dermatology 2004;209:228–229.
  59. Holder JE, Graham-Brown RAC, Camp RDR: Partial unilateral lentiginosis associated with blue naevi. Br J Dermatol 1994;130:390–393.
  60. Hofmann UB, Ogilvie P, Müllges W, Bröcker EB, Hamm H: Congenital unilateral speckled lentiginous blue nevi with asymmetric spinal muscular atrophy. J Am Acad Dermatol 1998;39:326–329.
  61. Ishibashi A, Kimura K, Kukita A: Plaque-type blue nevus combined with lentigo (nevus spilus). J Cutan Pathol 1990;17:241–245.
  62. Betti R, Inselvini E, Crosti C: Blue nevi and basal cell carcinoma within a speckled lentiginous nevus. J Am Acad Dermatol 1999;41:1039–1041.
  63. Betti R, Inselvini E, Palvarini M, Crosti C: Agminate and plaque-type blue nevus combined with lentigo, associated with follicular cyst and eccrine changes: a variant of speckled lentiginous nevus. Dermatology 1997;195:387–390.
  64. Guillot B, Bessis D, Barnéon G, Monpoint S, Guilhou JJ: Malignant melanoma occurring on a ‘naevus on naevus’. Br J Dermatol 1991;124:610–611.
  65. Rhodes AR, Mihm MC: Origin of cutaneous melanoma in a congenital dysplastic nevus spilus. Arch Dermatol 1990;126:500–505.
  66. Kurban RS, Preffer FI, Sober AJ, Mihm MC, Barnhill RL: Occurrence of melanoma in ‘dysplastic’ nevus spilus: report of case and analysis by flow cytometry. J Cutan Pathol 1992;19:423–428.
  67. Hofmann-Wellenhof R, Soyer HP, Smolle J, Kerl H: Spitz’s nevus arising on a nevus spilus. Dermatology 1994;189:265–268.
  68. Kinoshita K, Shinkai H, Utani A: Phacomatosis pigmentokeratotica without extracutaneous abnormalities. Dermatology 2003;207:415–416.
  69. Saraswat A, Dogra S, Bansali A, Kumar B: Phacomatosis pigmentokeratotica associated with hypophosphataemic vitamin D-resistant rickets: improvement in phosphate homeostasis after partial laser ablation. Br J Dermatol 2003;148:1074–1076.
  70. Selvaag E, Thune P, Larsen TE: Segmental neurofibromatosis presenting as a giant naevus spilus. Acta Derm Venereol 1994;74:327–334.
  71. Piqué E, Aguilar A, Fariña MC, Gallego MA, Escalonilla P: Partial unilateral lentiginosis: report of seven cases and review of the literature. Clin Exp Dermatol 1995;20:319–322.
  72. Kiene P, Brodersen JP, Fölster-Holst R: ‘Blaue’ Variante eines Naevus spilus. Hautarzt 1995;46:349–351.
  73. Töppe F, Haas N: Naevus spilus géant avec naevus bleus: à propos d’un cas. Ann Dermatol Venereol 1988;15:703–707.

    External Resources

  74. Marchesi L, Naldi L, Parma A, Locati F, Cainelli T: Agminate blue nevus combined with lentigo: a variant of speckled lentiginous nevus? Am J Dermatopathol 1993;15:162–165.
  75. Happle R: Loss of heterozygosity in human skin. J Am Acad Dermatol 1999;41:143–161.

Pay-per-View Options
Direct payment This item at the regular price: USD 38.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 26.50