Papillon-Lefevre Syndrome Sherry Lockhart: Date: 11/8/2010

Patient: Age: 1-4 years of age (on set) Gender: Male and female Height: Varies Weight: Varies Vital Signs: HR: Respiratory rate: Blood Pressure:1 10/70 mmHg Temperature: 98.7 F Chief complaint Gingival Inflammation, severe periodontitis, tooth mobility, halitosis, heavy plaque and calculus Medical Alert None Name of Syndrome:Papillon-Lefevre Syndrome (Palmoplantar Keratoderma with Periodontosis) Cause of Syndrome - Medical History (if applicable) -Inherited as an autosomal recessive trait -Mutation of the gene that produces the enzyme Cathespin C. -Greater frequency in consanguineous offspring

Orofacial Clinical Features -several or all of their teeth missing. -severe periodontal disease -heavy plaque and calculus -abscesses -foul odor in mouth -severe alveolar resorption -inflamed gingival tissue Age/race/sex predilections and Systemic Clinical Features: -Children are born looking completely normal. They may have redness on palms of hands and soles of feet. -Teeth erupt in normal sequence, position, and time. -At age 1, when primary teeth starting to erupt, the gum tissue is severely inflamed and generalized aggressive periodontitis accompany the teeth. -By age 4, the child has lost all of there primary dentition. -Gingival tissue in mouth goes back to healthy & normal. -Eruption of the permanent dentition begins at normal age and in normal sequence. -The same symptoms accompany these teeth. -Formation of abscesses and alveolar bone loss occur. -Patient will loose their permanent teeth and be completely edentulous by age Patients also suffer from palmoplantar keratosis -Patients have also suffered from erythematous hyperkeratosis plaques on their elbows and knees. -May also have pus-producing skin infections, abnormalities of nails, and excessive perspiration. Radiographic Features of this Syndrome -teeth seem to be “floating on air” -severe bone loss

Special considerations in Treatment of this patient? How is it Diagnosed? How common is it? Is it a horse or zebra? Etiology is still unknown—3 factors suggested: 1.Impairment of neutraphil chemotaxis, phagocytosis and bactericidal activities accompanied by a decrease in cell migration 2.Presence of virulent gram-negative anaerobic pathogens in periodontal plaque and periodontal pockets 3.Defect in immunity -reduced lymphocyte response -depression of helper T cells -deficient monocytic function -elevation in serum IgG -degenerative changes in plasma cells Treatment of this patient is critical -conventional periodontal therapy -oral hygiene instruction -systemic antibiotics ZEBRA Only 1-4 cases per million persons

Papillon-Lefevre Syndrome

Intraoral Pictures

List Sources and References (This should be more than your text book!) Works Cited Page Oral Pathology for the Dental Hygienist 5th Edition Ibsen & Phelan_2009 Pgs Dermatology Online Journal Volume 10 Number 1 “Papillon- Lefevre Syndrome: Case report and review of the literature”. “Children’s Health Papillion Lefevre Syndrome”. U.S. National Library of Medicine National Institutes of Health “The Papillon-Lefevre Syndrome: neutraphil dysfunction with severe periodontal disease”.