title: Palmoplantar keratoderma with progressive gingivitis and recurrent pyodermas.
abstract: Papillon-Lefèvre syndrome (PLS) is a rare inherited palmoplantar keratoderma (PPK) that is associated with progressive gingivitis and recurrent pyodermas. We present a case exhibiting classic features of this autosomal-recessive condition and review the current understanding of its pathophysiology, diagnosis, and treatment. Additionally, a review of pertinent transgredient PPKs is undertaken, with key and distinguishing features of each syndrome highlighted.
authors.forename: Tyler A
authors.initials: TA
authors.lastname: Moss
authors.affiliation: Department of Dermatology, Kimbrough Ambulatory Care Center, 2480 Llewellyn Ave, Ste 5800, Fort Meade, MD 20755-5129, USA. anne.p.spillane.mil@mail.mil.
authors.forename: Anne P
authors.initials: AP
authors.lastname: Spillane
authors.forename: Sam F
authors.initials: SF
authors.lastname: Almquist
authors.forename: Patrick E
authors.initials: PE
authors.lastname: McCleskey
authors.forename: Oliver J
authors.initials: OJ
authors.lastname: Wisco
mesh_terms: D000328:Adult; D020789:Cathepsin C / Q000235:genetics; D018450:Disease Progression; D005260:Female; D005891:Gingivitis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D009154:Mutation; D010214:Papillon-Lefevre Disease / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology*; D011711:Pyoderma / Q000209:etiology* / Q000473:pathology; D012008:Recurrence
chemical_list: C533918:CTSC protein, human; D020789:Cathepsin C