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)

Abstract

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].

Keywords

Dermatol Fami 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1992

Authors and Affiliations

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

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