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: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Health
2021-06-01
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| Series: | International Journal of Dermatology and Venerology |
| Online Access: | http://journals.lww.com/10.1097/JD9.0000000000000042 |
| Summary: | 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. |
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| ISSN: | 2096-5540 2641-8746 |