Abstract
The Xeroderma pigmentosum [XP] is a rare inherited skin disorder and transmitted in an autosomal recessive manner. The aim of the present article is to report a XP case with oral manifestations and to discuss the role of the dental professional management of this entity. A 5 year old male presented lentigos all over the skin and predominated in photoexposed areas. Oral hygiene was good and dental affection was important. No lesions were observed in the lips, tongue and the rest of the bucal mucosa but multiples teeth with cavities were observed. Antibiotic treatment was started and surgical treatment. Besides dermatological, ophtamological and neurological management, XP patients require constant dental care and follow –up in order to control the occurrence of new lesions on the lips or inside oral cavity.
Highlights
Xeroderma pigmentosum [XP] was first described by Hebra and Kaposi in 1874. 8 years later, in 1882 Moriz Kaposi coined the term “Xeroderma Pigmentosum” which translates to “dry parchment skin” inspired by its characteristic dry skin with lentigos [freckling of the skin] [1,2].The Xeroderma pigmentosum is a rare inherited skin disorder caused, in 80% of the patiens, by a defect in DNA nucleotide excision repair mechanisms [NER; DNA) and transmitted in an autosomal recessive manner
Ophtamological and neurological management, XP patients require constant dental care and follow –up in order to control the occurrence of new lesions on the lips or inside oral cavity
The XP is characterized by a defect in DNA nucleotide excision repair mechanisms (NER; DNA)
Summary
Xeroderma pigmentosum [XP] was first described by Hebra and Kaposi in 1874. 8 years later, in 1882 Moriz Kaposi coined the term “Xeroderma Pigmentosum” which translates to “dry parchment skin” inspired by its characteristic dry skin with lentigos [freckling of the skin] [1,2]. The Xeroderma pigmentosum is a rare inherited skin disorder caused, in 80% of the patiens, by a defect in DNA nucleotide excision repair mechanisms [NER; DNA) and transmitted in an autosomal recessive manner. It is characterized by photosensitivity, pigmentary changes, premature skin aging, and malignant tumor development. The clinical variant XP-C is the most founded in the United States, Europe and North of Africa, and, XP- subtype A is more frequent in Japan [2]. The incidence in the United States and Europe is one case per million habitants, in Africa and Japan, the incidence is ten times more elevated [5]. The aim of the present article is to report a XP case with oral manifestations and to discuss the role of the dental professional management of this entity
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