The Cutaneous Manifestations of the Familial Atypical Multiple Mole Melanoma (FAMMM) Syndrome
- 17 May 2019
- book chapter
- Published by Taylor & Francis
Abstract
The cutaneous manifestations of the familial atypical multiple mole melanoma (FAMMM) syndrome (hereditary dysplastic nevus syndrome — HDNS) have only been recognized since the middle of the 1970s by two different independent research groups. 1,2 This disorder has been established by several investigative teams 1–5 to be an autosomal dominantly inherited trait with incomplete penetrance, variable expressivity of phenotype, and heterogeneity of the genotype. The characteristic cutaneous findings in members of kindreds with the classical presentation of this disorder are (1) the occurrence of increased incidence of cutaneous malignant melanoma (CMM); (2) the members of the kindred can have an increased number of total body nevi with counts greater than normal; and (3) the presence of multiple large atypical nevi which are usually clinically distinguishable from normal nevi when greater than 4 mm in diameter. This disorder has gone through the usual evolution of new discoveries; that is, there is sudden enlightenment followed by an increasing insecurity of understanding of the pathogenesis, as new information accrues.This publication has 1 reference indexed in Scilit:
- Familial atypical multiple mole-melanoma syndrome.Journal of Medical Genetics, 1978