Biomedicine

Abstract

Xeroderma pigmentosum (XP) is a rare hereditary disease which is characterized by photosensitivity, abnormal pigmentations with freckling, actinic hyperkeratosis, scarring and prematureness of skin along with malignancies of ectodermal origin. The defect is in the normal DNA repair gene which causes the typical clinical manifestations depending on the degree of ultraviolet light exposure, especially in the sun-exposed areas of the skin and mouth. The disorder is often found among populations where marriage of close blood relatives is common. Oral manifestations usually present as actinic cheilitis, and squamous cell carcinoma of lip and tongue. Here we report two cases of xeroderma pigmentosum with oral findings as generalized aggressive periodontitis which is a rare manifestation.