Genodermatoses are an inherited skin disorder often grouped into three categories: chromosomal, single gene, and polygenetic. Several of these disorders are isolated and also have an oral phenomenon, called Oral Genodermatoses. Between this Xeroderma pigmentosum is a rare genodermatosis as well as rare autosomal recessive disease in which excessive ultraviolet radiation causes skin, ocular, neurological, and oral lesions along with the development of cutaneous and internal malignancies at an early age. There is no definitive cure for the disease. Avoidance of ultraviolet radiation, use of protective clothing, sunscreens, oral retinoid, and 5-fluorouracil and regular consultations with dermatologists, ophthalmologists, neurologists and dentists forms an important part of the treatment protocol. This paper aims to throw light on the etiopathogenesis, clinical features and treatment modalities of this life-threatening disease. There is also a special mention of the oral manifestations and dental health considerations of the rare disorder.
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