Genodermatoses with Malignant Potential

Genodermatoses with malignant potential represent a heterogeneous group of inherited skin disorders associated with an elevated risk of developing cutaneous and extracutaneous malignancies. These conditions are often characterized by distinctive cutaneous features that provide important diagnostic clues and allow for early recognition. The predisposition to cancer in these disorders arises from underlying genetic mutations that disrupt pathways regulating DNA repair, cell cycle control, and genomic stability. Recognizing the malignant potential of these genodermatoses is crucial, as vigilant surveillance, timely diagnosis, and appropriate interventions can substantially improve patient outcomes.Only the malignancy aspect will be reviewed for conditions discussed in detail in other chapters.