Hereditary photodermatoses.

ABSTRACT

Photodermatoses are defined as the abnormal reactions of the skin to photons, usually those of wavelengths found in sunlight. These reactions can be caused by a wide variety of reasons, including defects in repair oflight-induced DNA lesions, the interaction of certain chemicals or medications with sunlight to produce toxic mediators and photo-induced immune reactions. In this chapter we will describe photodermatoses that are associated with hereditary conditions. These can be subdivided into several groups dermatoses caused by abnormal metabolic conditions, idiopathic photodermatoses, defects in cancer suppressor genes not directly involved in DNA repair but that predispose to photodistributed tumors and photosensitivity due to abnormalities in DNA repair pathways. Special emphasis will be placed on the relatively recently described UV-sensitive syndrome.