Self-reported clinical features and treatment effectiveness of Papillon–Lefèvre syndrome patients from five Latin American countries: A cross-sectional online survey study

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dc.coverageDOI: 10.1111/ajd.14219
dc.creatorAlfaro-Sepúlveda, Daniela
dc.creatorSalinas, Pía Parra
dc.creatorValenzuela, Fernando
dc.creatorGonzalez, Carmen Gloria
dc.creatorBurckhardt-Bravo, Valentina
dc.creatorFerrari-Sande, Dominga
dc.date2024
dc.date.accessioned2026-01-05T21:09:22Z
dc.date.available2026-01-05T21:09:22Z
dc.description<p>Background/Objectives: Most studies about Papillon–Lefèvre syndrome (PLS) are limited to case reports and patients of the same nationality. This study aimed to determine the self-reported prevalence of signs, symptoms and treatment effectiveness in PLS patients from five Latin American countries. Methods: An online survey was conducted among adult and paediatric patients from Mexico, Argentina, Colombia and Brazil. Data were collected using multiple-choice, open-ended and image-chooser questions on demographics, signs and symptoms, perceived treatment effectiveness and quality of life. Results: Seventeen patients (10 males and 7 females) aged 4–47 years were surveyed. All had palmoplantar hyperkeratosis. Other affected sites were the feet and hand dorsum (82.35%), Achilles tendon (88.24%), forearms (58.82%), legs (29.41%) and glutes (23.53%). They frequently presented hyperhidrosis and nail pitting. Four had a history of delayed umbilical cord separation. All used topical treatments, with moderate effectiveness; half used oral retinoids, perceived as highly effective. Most reported decreased quality of life and walking difficulties. Conclusions: The study's results align with prior research on PLS, but reveal new insights, including the impact on patients' quality of life and a history of delayed umbilical cord separation. These findings warrant consideration in future research and patient care.</p>eng
dc.identifierhttps://investigadores.uandes.cl/en/publications/0d9d47fe-3844-4810-b62a-e40c28d19461
dc.identifier.urihttps://repositorio.uandes.cl/handle/uandes/63832
dc.languageeng
dc.rightsinfo:eu-repo/semantics/restrictedAccess
dc.sourcevol.65 (2024) nr.4 p.305-310
dc.subjectcathepsin C
dc.subjectgenodermatosis
dc.subjectpalmoplantar hyperkeratosis
dc.subjectPapillon–Lefèvre syndrome
dc.subjectperiodontitis
dc.titleSelf-reported clinical features and treatment effectiveness of Papillon–Lefèvre syndrome patients from five Latin American countries: A cross-sectional online survey studyeng
dc.typeArticleeng
dc.typeArtículospa
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