Genodermatoses with malignant potential.

PURPOSE OF REVIEW: The delineation of syndromes carrying a predisposition to malignancy has led to great insights into the molecular biology of malignancy. Many such syndromes have cutaneous findings which can precede the development of neoplasia. Early recognition of the cutaneous stigmata of the genodermatoses with malignant potential can lead to early diagnosis and initiation of proper screening and treatment when indicated.

RECENT FINDINGS: This article reviews 'classic' genodermatoses with malignant potential and highlights recent recommendations for screening and treatment. Additionally more recently delineated syndromes and their cutaneous findings are discussed.

SUMMARY: Certain inherited syndromes with a risk of neoplasia exhibit characteristic cutaneous findings. Recognition of these findings by the astute practitioner can lead to early intervention which can impact the course of these rare diseases.