{Reference Type}: Journal Article
{Title}: Self-reported clinical features and treatment effectiveness of Papillon-Lefèvre syndrome patients from five Latin American countries: A cross-sectional online survey study.
{Author}: Alfaro-Sepúlveda D;Salinas PP;Valenzuela F;Gonzalez CG;Burckhardt-Bravo V;Ferrari-Sande D;
{Journal}: Australas J Dermatol
{Volume}: 65
{Issue}: 4
{Year}: 2024 Jun 13
{Factor}: 2.481
{DOI}: 10.1111/ajd.14219
{Abstract}: <B>OBJECTIVE: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.