Genodermatoses (Inherited Diseases with Cutaneous Manifestations): Molecular Biology and Diagnosis

Genodermatoses are a variable group of inherited diseases that initially, or at least very early in life, present with cutaneous findings. They include divergent diseases, which could be divided into, for instance, disorders of pigmentation (example - albinism); disorders of keratinization (example - congenitalichthyosi form erythroderma), etc. The focus of this article is a subset of genodermatoses that are associated with increased risk of various skin neoplasms that develop either very early or later in life. Thus, this subset has often been dubbed as "inherited or heritable cancer syndromes". However, it is important to realize that not all of inherited cancer syndromes have protean skin manifestations, and that indeed, not all of the genodermatoses have neoplastic associations. The particular diseases this article deals with are Muir-Torre syndrome, Cowden\u27s disease, and Carney\u27s complex