Papillon–Lefevre syndrome: Reporting consanguinity as a risk factor
Identifieur interne : 005317 ( Ncbi/Curation ); précédent : 005316; suivant : 005318Papillon–Lefevre syndrome: Reporting consanguinity as a risk factor
Auteurs : Aasim Farooq Shah [Inde] ; Pradeep Tangade [Inde] ; Swatantra Agarwal [Inde]Source :
- The Saudi Dental Journal [ 1013-9052 ] ; 2014.
Abstract
Papillon–Lefevre syndrome (PLS) is an autosomal recessive genetic disorder characterized by palmoplantar hyperkeratosis associated with severe early-onset periodontitis and premature loss of primary and permanent teeth. This report describes two cases of PLS in 28-year-old female and 16-year-old male siblings with consanguineously married parents. The patients presented to the Department of Public Health Dentistry of a dental education and research institute in India with thickening, flaking, and scaling of the skin on the palms and soles of the feet. On oral examination, the female patient presented completely resorbed maxillary and mandibular alveolar ridges with retention of only the third molars. The male patient retained only teeth 18, 13, 28, 38, and 45. Based on complete histories and clinical examination findings, a final diagnosis of PLS was made and treatment was initiated using an interdisciplinary dental approach in both cases.
Url:
DOI: 10.1016/j.sdentj.2014.02.004
PubMed: 25057233
PubMed Central: 4095039
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sortKey="Shah, Aasim Farooq" sort="Shah, Aasim Farooq" uniqKey="Shah A" first="Aasim Farooq" last="Shah">Aasim Farooq Shah</name><affiliation wicri:level="1"><nlm:aff id="af005">Department of Public Health Dentistry, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad 244001, Uttar Pradesh, India</nlm:aff><country xml:lang="fr">Inde</country><wicri:regionArea>Department of Public Health Dentistry, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad 244001, Uttar Pradesh</wicri:regionArea><wicri:noRegion>Uttar Pradesh</wicri:noRegion></affiliation></author><author><name sortKey="Tangade, Pradeep" sort="Tangade, Pradeep" uniqKey="Tangade P" first="Pradeep" last="Tangade">Pradeep Tangade</name><affiliation wicri:level="1"><nlm:aff id="af005">Department of Public Health Dentistry, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad 244001, Uttar Pradesh, India</nlm:aff><country xml:lang="fr">Inde</country><wicri:regionArea>Department of Public Health Dentistry, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad 244001, Uttar Pradesh</wicri:regionArea><wicri:noRegion>Uttar Pradesh</wicri:noRegion></affiliation></author><author><name sortKey="Agarwal, Swatantra" sort="Agarwal, Swatantra" uniqKey="Agarwal S" first="Swatantra" last="Agarwal">Swatantra Agarwal</name><affiliation wicri:level="1"><nlm:aff id="af010">Department of Prosthodontics, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad 244001, Uttar Pradesh, India</nlm:aff><country xml:lang="fr">Inde</country><wicri:regionArea>Department of Prosthodontics, Kothiwal Dental College & Research Centre, Kanth Road, Moradabad 244001, Uttar Pradesh</wicri:regionArea><wicri:noRegion>Uttar Pradesh</wicri:noRegion></affiliation></author></analytic><series><title level="j">The Saudi Dental Journal</title><idno type="ISSN">1013-9052</idno><idno type="eISSN">1658-3558</idno><imprint><date when="2014">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Papillon–Lefevre syndrome (PLS) is an autosomal recessive genetic disorder characterized by palmoplantar hyperkeratosis associated with severe early-onset periodontitis and premature loss of primary and permanent teeth. This report describes two cases of PLS in 28-year-old female and 16-year-old male siblings with consanguineously married parents. The patients presented to the Department of Public Health Dentistry of a dental education and research institute in India with thickening, flaking, and scaling of the skin on the palms and soles of the feet. On oral examination, the female patient presented completely resorbed maxillary and mandibular alveolar ridges with retention of only the third molars. The male patient retained only teeth 18, 13, 28, 38, and 45. Based on complete histories and clinical examination findings, a final diagnosis of PLS was made and treatment was initiated using an interdisciplinary dental approach in both cases.</p></div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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