Increased Number of Multiple Melanomas in Sporadic and Familial Variants of Dysplastic Naevus Syndrome

  • Christian Sigg
Conference paper
Part of the ESO Monographs book series (ESO MONOGRAPHS)


In the past decade, a new cancer-associated genodermatosis has been defined, the socalled Dysplastic Naevus Syndrome (DNS) or familial atypical multiple mole melanoma syndrome (FAMMM) [1,2]. This dominant trait with extensive heterogeneity and variable expression shows clinical and histological features characterised by its name: FAMMM is characterised by the association of melanomas affecting several members of a family and the presence of dysplastic naevi. Apart from the inherited variant of DNS there also exists a sporadic form: the frequency of sporadic dysplastic naevi appears to be 2–7% in white adults in the USA. In the Swiss population, DNS was found in 2.5% of men aged 20 and in 7% of patients attending dermatological clinics because of pigmented moles [3,4]. Remarkably, in a study including 939 schoolchildren, clinically defined dysplastic naevi were observed in 2.7%; although histological examination did not confirm all conditions for dysplastic naevus, the melanonae-vocytic status of these children (fair skin complexion, increased total number of acquired naevi and increased number of irregular naevi) is strongly suggestive of an early stage of DNS in childhood [5].


Familial Melanoma Familial Variant Familial Atypical Multiple Mole Melanoma Multiple Melanoma Dysplastic Naevus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Clark W, Reimer R, Greene R, Ainsworth A, Mastrangelo M: Origin of familial malignant melanoma cases from hereditable melanocytic lesions. Arch Dermatol 1978 (114):732–738PubMedCrossRefGoogle Scholar
  2. 2.
    Lynch H, Frichot B, Lynch J: Familial atypical multiple mole-melanoma syndrome. J Med Genet 1978(15):352–356PubMedCrossRefGoogle Scholar
  3. 3.
    Sigg C, Pelloni F: Frequency of acquired melanonevocytic nevi and their relationship to skin complexion in 939 schoolchildren. Dermatologica 1989 (179) 123–128PubMedCrossRefGoogle Scholar
  4. 4.
    Sigg C, Pelloni F: Hautstatus und Häufigkeit von Dermatosen bei 603 Rekruten der schweizer Armee. Schweiz Z Milit Med 1990 (67):23–28Google Scholar
  5. 5.
    Sigg C, Pelloni F, Schnyder UW: Increased number of acquired melanonevocytic nevi and atypical appearance of nevi in children. Possible indications of the early stage of dysplastic nevus syndrome in childhood (in press)Google Scholar
  6. 6.
    Lynch HT, Fusaro RM, Pester J, Oosterhuis JA, Went LN, Rumke P, Neering H, Lynch JF: Tumor spectrum in the FAMMM syndrome. Br J Cancer 1981 (44):533–540CrossRefGoogle Scholar
  7. 7.
    Lynch HT, Frichot BC, Lynch P, Lynch JF, Guirgis HA: Family studies of malignant melanoma and associated cancer. Surg Gynecol Obstet 1975 (141):517–522PubMedGoogle Scholar
  8. 8.
    Sigg C, Pelloni F: Dysplastic nevi and germ cell tumors of the testis -a possible further tumor in the spectrum of associated malignancies in dysplastic nevus syndrome. Dermatologica 1988 (176):109–110PubMedCrossRefGoogle Scholar
  9. 9.
    Sigg C: Increased number of DNS and other disturbances of melanonevocytic system in infertile men (in preparation)Google Scholar
  10. 10.
    Sigg C, Pelloni F, Schnyder UW: Gehäufte Mehrfachmelanome bei sporadischem und familiärem dysplastischen Nävuszellnävus-Syndrom. Hautarzt 1989 (40):548–552PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1992

Authors and Affiliations

  • Christian Sigg
    • 1
  1. 1.Institute of DermatohistopathologyZürichSwitzerland

Personalised recommendations