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Papillon-Lefèvre syndrome: Case report and review of the literature
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2004
Year
15-Year-old BoyPathologyDermatologyOrthopaedic SurgeryPathologic LesionPapillon-lefèvre SyndromeHuman PathologySurgical PathologyPleural DiseaseFriable GumsHistopathologyDermatopathologySclerodermaCase ReportThumb HypoplasiaVeterinary ScienceClinical PathologyMedicineConnective Tissue Disease
A 15-year-old boy presented with symmetric, well-demarcated, yellowish, keratotic plaques over the skin of his palms and soles extending onto the dorsal surfaces. Well-circumscribed, psoriasiform, erythematous, scaly plaques were also present on the elbows and knees bilaterally along with dystrophy an transverse grooving of the nails. He also had swollen and friable gums since the age of 3 with subsequent loss of most of his permanent dentition. These findings are consistent with Papillon-Lefèvre syndrome. The clinical presentation, differential diagnosis, complications and management of this syndrome are discussed.