Transcript Case Study - LCCCOralPathII

Papillon-Lefevre Syndrome
Sherry Lockhart:
Date: 11/8/2010
Name of Syndrome:
Papillon-Lefevre Syndrome
Patient:
Age:
1-4 years of age (on set)
Gender:
Male and female
Height:
Varies
Weight:
Varies
Vital Signs:
HR: 60-100
Respiratory rate: 12-20
Blood Pressure:1 10/70 mmHg
Temperature: 98.7 F
(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.
Chief
complaint
Medical
Alert
Gingival Inflammation, severe periodontitis,
tooth mobility, halitosis, heavy plaque and
calculus
None
-Greater frequency in consanguineous offspring
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.
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
-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.
Radiographic Features of this Syndrome
-The same symptoms accompany these teeth.
-teeth seem to be “floating on air”
-severe bone loss
-Formation of abscesses and alveolar bone loss occur.
-Patient will loose their permanent teeth and be completely
edentulous by age 14-17.
-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.
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 209-210
www.dermatology.cdib.org/.../khachemoune.html Dermatology Online Journal Volume 10 Number 1 “PapillonLefevre Syndrome: Case report and review of the literature”.
http://children.webmd.com/papollon-lefevre-syndrome “Children’s Health Papillion Lefevre Syndrome”.
www.PubMed.gov U.S. National Library of Medicine National Institutes of Health “The Papillon-Lefevre
Syndrome: neutraphil dysfunction with severe periodontal disease”.