Author/Authors :
Miri-Aliabad ، Ghasem Department of Pediatrics - Children and Adolescent Health Research Center - Zahedan University of Medical Sciences , Asgarzadeh ، Leila Department of Pediatrics - Tabriz University of Medical Sciences
Abstract :
Xeroderma pigmentosum (XP) is a rare genetic disorder inherited in an autosomal recessive pattern. Patients with XP are extremely sensitive to ultraviolet (UV) radiation that leads to defective DNA repair. People with XP often suffer from problems in the eyes, face, neck, and other areas of the body, frequently exposed to sunlight. It is characterized by photosensitivity, dry skin, pigmentary changes of the skin, premature skin aging, and a considerable increase in incidence rates of malignant skin tumors. There is no cure for XP. In this article, we have described four patients from two families, three of whom had malignant skin tumors.
Keywords :
Xeroderma Pigmentosum , Photosensitivity , Melanoma , DNA Repair