Papillon-Lefèvre Syndrome: A Case Report
Abstract. Introduction:. Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder characterized by a diffuse palmoplantar hyperkeratosis and early periodontal destruction. Only a few PLS cases are reported by dermatological clinicians due to its rarity. Here, we reported a 16-year-old Chin...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Health
2021-06-01
|
| Series: | International Journal of Dermatology and Venerology |
| Online Access: | http://journals.lww.com/10.1097/JD9.0000000000000042 |
| _version_ | 1818648291640868864 |
|---|---|
| author | Yu-Lei Zhao Zhao Feng Bin Hu Liu-Qing Chen |
| author_facet | Yu-Lei Zhao Zhao Feng Bin Hu Liu-Qing Chen |
| author_sort | Yu-Lei Zhao |
| collection | DOAJ |
| description | Abstract. Introduction:. Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder characterized by a diffuse palmoplantar hyperkeratosis and early periodontal destruction. Only a few PLS cases are reported by dermatological clinicians due to its rarity.
Here, we reported a 16-year-old Chinese boy presented with diffuse transgradient palmoplantar hyperkeratosis since 4 years of age.
Case presentation:. The patient had also experienced recurrent episodes of swollen gums with premature loss of teeth. He is treated with multidisciplinary approaches. On follow-up, he continued to have recurrent gingival inflammation.
Discussion:. The etiopathogenesis of PLS is obscure and its management presents a special challenge, which call for in-depth studies that will reveal the complex interactions of genetic, immunologic, and microbiological factors involved in pathogenesis of PLS.
Conclusion:. PLS is a very rare genodermatosis with characterized palmoplantar hyperkeratosis. Dermatological clinicians should be aware of this rare clinical entity to promote its early diagnosis. |
| first_indexed | 2024-12-17T01:16:06Z |
| format | Article |
| id | doaj.art-0c41e143478249448a6f25372913700f |
| institution | Directory Open Access Journal |
| issn | 2096-5540 2641-8746 |
| language | English |
| last_indexed | 2024-12-17T01:16:06Z |
| publishDate | 2021-06-01 |
| publisher | Wolters Kluwer Health |
| record_format | Article |
| series | International Journal of Dermatology and Venerology |
| spelling | doaj.art-0c41e143478249448a6f25372913700f2022-12-21T22:08:59ZengWolters Kluwer HealthInternational Journal of Dermatology and Venerology2096-55402641-87462021-06-0142979910.1097/JD9.0000000000000042202106000-00008Papillon-Lefèvre Syndrome: A Case ReportYu-Lei ZhaoZhao FengBin HuLiu-Qing ChenAbstract. Introduction:. Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder characterized by a diffuse palmoplantar hyperkeratosis and early periodontal destruction. Only a few PLS cases are reported by dermatological clinicians due to its rarity. Here, we reported a 16-year-old Chinese boy presented with diffuse transgradient palmoplantar hyperkeratosis since 4 years of age. Case presentation:. The patient had also experienced recurrent episodes of swollen gums with premature loss of teeth. He is treated with multidisciplinary approaches. On follow-up, he continued to have recurrent gingival inflammation. Discussion:. The etiopathogenesis of PLS is obscure and its management presents a special challenge, which call for in-depth studies that will reveal the complex interactions of genetic, immunologic, and microbiological factors involved in pathogenesis of PLS. Conclusion:. PLS is a very rare genodermatosis with characterized palmoplantar hyperkeratosis. Dermatological clinicians should be aware of this rare clinical entity to promote its early diagnosis.http://journals.lww.com/10.1097/JD9.0000000000000042 |
| spellingShingle | Yu-Lei Zhao Zhao Feng Bin Hu Liu-Qing Chen Papillon-Lefèvre Syndrome: A Case Report International Journal of Dermatology and Venerology |
| title | Papillon-Lefèvre Syndrome: A Case Report |
| title_full | Papillon-Lefèvre Syndrome: A Case Report |
| title_fullStr | Papillon-Lefèvre Syndrome: A Case Report |
| title_full_unstemmed | Papillon-Lefèvre Syndrome: A Case Report |
| title_short | Papillon-Lefèvre Syndrome: A Case Report |
| title_sort | papillon lefevre syndrome a case report |
| url | http://journals.lww.com/10.1097/JD9.0000000000000042 |
| work_keys_str_mv | AT yuleizhao papillonlefevresyndromeacasereport AT zhaofeng papillonlefevresyndromeacasereport AT binhu papillonlefevresyndromeacasereport AT liuqingchen papillonlefevresyndromeacasereport |