Familial keratoses of actinic distribution associated with internal malignancy and cellular hypersensitivity to UVA

In members of a family there appeared to be an association between the development of cutaneous pigmented keratoses in sun-exposed sites, and the later evolution of internal malignancies, particularly carcinoma of the uterus. Affected individuals were not clinically photosensitive, but their fibroblasts demonstrated gross cytopathic changes, low survival indices and an increased frequency of DNA single strand breaks following exposure to long-wave ultraviolet radiation (UVA). These clinical and cellular features appear to identify an unrecognized syndrome that may not be uncommon. Recognition of this syndrome in a family may prevent certain malignancies developing in those affected.