ABSTRACT
The cutaneous manifestations of the familial atypical multiple mole melanoma (FAMMM) syndrome have only been recognized since the middle of the 1970s by different independent research groups. The genetic trait of the FAMMM syndrome has been present for eons, but its failure to be recognized by dermatologists and oncologists is probably due to our historical lack of acceptance of the extent of hereditary cancer. To the informed dermatologist, the classic florid cutaneous expressions of the FAMMM syndrome are easily recognized at a glance without the slightest hesitation. The histology of the atypical nevus taken from FAMMM patients who have classic lesions shows cellular melanocytic atypia, architectural atypia, dermal fibrosis, angioneogenesis, and chronic lymphocytic inflammatory infiltrate. The precise definition of histologic dysplasia for the confirmation of the clinical diagnosis of atypical nevi is evolving, and its lower limits remain controversial.
