Papillon-Lefevre Syndrome in a Family: A Simultaneous Occurrence in Multiple Siblings

Background: Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis, aggressive periodontitis, and premature exfoliation of both deciduous and permanent teeth. Due to severe alveolar bone resorption, teeth are often lost two to three years after eruption, and affected individuals typically become edentulous by the age of 16-17.Case report: This study presents three siblings from the same family, each exhibiting different clinical manifestations of PLS. The first case is a 12-year-old girl who was referred to the Department of Oral Medicine with complaints of tooth mobility. She was diagnosed with severe periodontitis, permanent tooth mobility, and palmoplantar hyperkeratosis. The second case is her 10-year-old brother presenting with similar symptoms, and the third is their 9-month-old sister who had signs of palmoplantar hyperkeratosisConclusion: PLS requires early diagnosis and multidisciplinary management. Timely diagnosis and treatment can help prevent the rapid progression of disease-related complications.

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Publication Details

Journal
SHILAP Revista de lepidopterología
Published
2025-05-01
DOI
https://doi.org/10.22038/jmds.2025.26036
Primary Topic
Oral microbiology and periodontitis research
Type
article
Field-Weighted Citation Impact
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article

Papillon-Lefevre Syndrome in a Family: A Simultaneous Occurrence in Multiple Siblings

SHILAP Revista de lepidopterología
Oral microbiology and periodontitis research
article

Papillon-Lefevre Syndrome in a Family: A Simultaneous Occurrence in Multiple Siblings

article en

Abstract

Background: Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis, aggressive periodontitis, and premature exfoliation of both deciduous and permanent teeth. Due to severe alveolar bone resorption, teeth are often lost two to three years after eruption, and affected individuals typically become edentulous by the age of 16-17.Case report: This study presents three siblings from the same family, each exhibiting different clinical manifestations of PLS. The first case is a 12-year-old girl who was referred to the Department of Oral Medicine with complaints of tooth mobility. She was diagnosed with severe periodontitis, permanent tooth mobility, and palmoplantar hyperkeratosis. The second case is her 10-year-old brother presenting with similar symptoms, and the third is their 9-month-old sister who had signs of palmoplantar hyperkeratosisConclusion: PLS requires early diagnosis and multidisciplinary management. Timely diagnosis and treatment can help prevent the rapid progression of disease-related complications.

SHILAP Revista de lepidopterología
Shahid Sadoughi University of Medical Sciences and Health Services (IR)
Good health and well-being
Openalex Percentile: Top 47%
Oral microbiology and periodontitis research
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