has part has part has_part realized in realized in metabolic process disease course Primary immunodeficiency due to a defect in innate immunity Malformation syndrome with skin/mucosae involvement Genetic malformation syndrome with odontal and/or periodontal component Autosomal recessive disease with diffuse palmoplantar keratoderma as a major feature Papillon-Lefèvre syndrome orphanet Papillon-Lefèvre syndrome (PLS) is a rare ectodermal dysplasia characterized by palmoplantar keratoderma associated with early-onset periodontitis. Keratosis palmoplantar - periodontopathy ICD10:Q82.8 MESH:D010214 OMIM:245000 PLS UMLS:C0030360 Papillon-Lefevre Disease Papillon Lefevre syndrome DOID:3389 Papillon-Lefvre syndrome (disorder)