Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma
Papillon–Lefevre syndrome is an autosomal recessive disorder distinguished mainly by a triad of palmar-plantar hyperkeratosis, aggressive periodontitis involving both deciduous and permanent dentition and intracranial calcifications. Patients also exhibit increased susceptibility to bacterial infect...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-12-01
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| Series: | Indian Journal of Dental Sciences |
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| Online Access: | https://journals.lww.com/10.4103/ijds.ijds_25_23 |
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| author | Manjiri Uttam Joshi Mukta Vijaykumar Vanjani Manali Jagtap |
| author_facet | Manjiri Uttam Joshi Mukta Vijaykumar Vanjani Manali Jagtap |
| author_sort | Manjiri Uttam Joshi |
| collection | DOAJ |
| description | Papillon–Lefevre syndrome is an autosomal recessive disorder distinguished mainly by a triad of palmar-plantar hyperkeratosis, aggressive periodontitis involving both deciduous and permanent dentition and intracranial calcifications. Patients also exhibit increased susceptibility to bacterial infections. Most of the cases are reported on account of homozygous mutations affecting cathepsin C (CTSC) gene, as both the alleles of cathepsin C gene are mutated. Periodontal disease can, however, develop either as a principal manifestation or be fostered by the presence of local factors. This article presents such a rare case report along with dermatological manifestations and its clinical significance from dental perspective. This will aid in a proper multidisciplinary treatment approach. |
| format | Article |
| id | doaj-art-83a1530aba034d4ba6fb283250e43862 |
| institution | Kabale University |
| issn | 0976-4003 2231-2293 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Dental Sciences |
| spelling | doaj-art-83a1530aba034d4ba6fb283250e438622025-01-07T06:10:33ZengWolters Kluwer Medknow PublicationsIndian Journal of Dental Sciences0976-40032231-22932024-12-0116420120410.4103/ijds.ijds_25_23Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic DilemmaManjiri Uttam JoshiMukta Vijaykumar VanjaniManali JagtapPapillon–Lefevre syndrome is an autosomal recessive disorder distinguished mainly by a triad of palmar-plantar hyperkeratosis, aggressive periodontitis involving both deciduous and permanent dentition and intracranial calcifications. Patients also exhibit increased susceptibility to bacterial infections. Most of the cases are reported on account of homozygous mutations affecting cathepsin C (CTSC) gene, as both the alleles of cathepsin C gene are mutated. Periodontal disease can, however, develop either as a principal manifestation or be fostered by the presence of local factors. This article presents such a rare case report along with dermatological manifestations and its clinical significance from dental perspective. This will aid in a proper multidisciplinary treatment approach.https://journals.lww.com/10.4103/ijds.ijds_25_23aggressive periodontitisdermatological manifestationskeratotic patchespalmoplantar hyperkeratosispapillon–lefevre syndrome |
| spellingShingle | Manjiri Uttam Joshi Mukta Vijaykumar Vanjani Manali Jagtap Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma Indian Journal of Dental Sciences aggressive periodontitis dermatological manifestations keratotic patches palmoplantar hyperkeratosis papillon–lefevre syndrome |
| title | Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma |
| title_full | Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma |
| title_fullStr | Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma |
| title_full_unstemmed | Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma |
| title_short | Keratosis Palmoplantar-periodontopathy Syndrome – A Clinical Diagnostic Dilemma |
| title_sort | keratosis palmoplantar periodontopathy syndrome a clinical diagnostic dilemma |
| topic | aggressive periodontitis dermatological manifestations keratotic patches palmoplantar hyperkeratosis papillon–lefevre syndrome |
| url | https://journals.lww.com/10.4103/ijds.ijds_25_23 |
| work_keys_str_mv | AT manjiriuttamjoshi keratosispalmoplantarperiodontopathysyndromeaclinicaldiagnosticdilemma AT muktavijaykumarvanjani keratosispalmoplantarperiodontopathysyndromeaclinicaldiagnosticdilemma AT manalijagtap keratosispalmoplantarperiodontopathysyndromeaclinicaldiagnosticdilemma |