Term
Another name for Hereditary Hemorrhagic Telangiectasia (HHT) |
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Definition
Osler-Weber-Rendu Syndrome |
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Term
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Definition
Rare, genetic disorder that affects blood vessels; autosomal dominant disorder; Affects 2-19/100,000 |
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Term
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Definition
Mutation in a gene known as endoglin, located on chrom 9. Frequently associated w/ pulmonary and/or cerebra; arteriovenous malformations |
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Definition
Due to gene on chrom 12 known as ALK1 |
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Term
What are HHT-1 and HHT-2 associated w/? |
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Definition
Nosebleeds (epistaxis) and telangiectases |
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Term
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Definition
Most dev telangiectasias (red spots formed by dilated blood vessels) often found on lips, tongue, fingers, or toes by age 30 |
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Term
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Definition
Manage complications, no way to prevent disease due to genetic nature; dentistry: prophylactic antibiotics prior to dental work if pt has lung AVM - request medical consult; avoid NSAIDs |
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Definition
5% die from brain abscess exhibiting oral microflora |
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Term
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Definition
Chronic, life-long incurable disease
T-cell mediated autoimmune disease
Characterized by exacerbation and remission
Treatment is empirical, usually immunomodulating
Regular dental exams and excellent OH are critical
Will always require followup
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Term
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Definition
Idiopathic, multifactoral including drugs, dental materails, hep C, GVHD; immunopathogenesis involving T cells; immunologically mediated/autoimmune |
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Term
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Definition
Occurs in 1-2% of population; same incidence as vitiligo, psoriasis, alopecia areata; world-wide distribution; usually develops in 5-6th decade of life; may occur in children; more common in women than men
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Term
Mucocutaneous clinical features of LP |
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Definition
20-30% skin only
12-50% skin and oral
30-50% oral only *More erosions and symptoms than those w/ only skin or both skin and oral
Less common - genital mucosa; vulvovaginal gingival syndrome 19%
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Term
Distribution of OLP (decreasing freq) |
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Definition
Buccal mucosa, tongue, gingiva, palate, floor of mouth, labial mucosa |
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Term
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Definition
Establish diagnosis:
- Clinical exam only - reticular, bilateral, asympatomatic
- Biopsy unilateral, painful, erosive
- Biopsy multiple/high risk sites for H&E
- Biopsy "gingival lesions only cases" - H&E, DIF
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Term
Differential diagnosis of OLP |
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Definition
Leukoplakia/epithelial dysplasia; desquamative gingival lesions; Lupus erythematosus; chronic ulcerative stomatitis |
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Term
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Definition
NSAIDs, Antihypertensive agents (angiotensin-converting enzyme inhibitors, Thiazides, diuretics, beta-blockers, calcium channel blockers), antimalarials, systemic gold |
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Term
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Definition
Reticular (90%), plaque, papular, atrophic (5-44%), erosive (9-46%), bullous
- May have more than one type of lesion at any one time
- May dev diff lesions over time |
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Term
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Definition
Elimination of irritants/xerostomia
Modification of reversible lichenoid reactions (drugs, dental restorations)
Therapy (topical, systemic)
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Term
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Definition
Topical therapy for biopsy confirmed painful or ulcerative disease
Corticosteroids, retinoids, tacrolimus, azathioprine, cyclosporine |
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Term
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Definition
- Directed towards painful, erythematous lesions
- Asymptomatic, reticular lesions should be left untreated unless gingival lesions complicate periodontal health
- No single, universally effective agent
- Multiple agents often necessary
- Treatment is palliative and non-curative
- Improvement is always temporary
- Requires long-term therapy w/ monitoring for adverse effects
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Term
Malignant transformation of OLP |
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Definition
- 1-2% incidence of oral SSCA
- Often involves tongue
- Regular followup and repeat biopsies of persistent firm, indurated, exophytic lesions
- Eliminate use of tobacco and alcohol
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Term
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Definition
Consider removal of dental restoration(s) if lesions:
- Develop following placement
- Are located adj to restorations |
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Term
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Definition
- Non-curable, chronic disease
- Asympatomatic lichen planus requires diagnosis and life-long followup due to risk of malignancies
- If symptomatic, accurate diagnosis will direct treatment (ex. erosive type, superimposed candidiasis, SCCA)
- A variety of treatments, usually immunomodulating, may be required over a lifetime
- Regular dental treatment w/ excellent oral hygiene is critical, especially w/ gingival lesions or in patients with dry mouth
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Term
Pathogenesis of Pemphigus Vulgaris |
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Definition
Circulating IgG autoantibodies to desmosomes of stratified squamous epithelium |
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Term
Definition of Pemphigus Vulgaris |
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Definition
Chronic autoimmune disease of unknown etiology; mucocutaneous; oral mucosa often precedes skin; usually middle aged adults |
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Term
Signs of Pemphigus vulgaris |
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Definition
Nikolsky's sign: minor trauma induces blister
Asboe-Hansen sign: blister will spread by pressing on it |
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Term
Clinical features of pemphigus vulgaris |
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Definition
- Blisters are superficial
- Oral ulcers are almost always very painful
- Interfere w/ eating and OH
- Excessive salivation, may be blood tinged
- Oral malodor (halitosis)
- Biopsy site slow to heal
- Oral ulcers heal without scarring
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Term
Intraoral sites of pemphigus vulgaris |
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Definition
Soft palate, buccal mucosa, tongue, gingiva |
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Term
True or false: Pemphigus vulgaris has variants. |
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Definition
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Term
Differential diagnosis of pemphigus vulgaris |
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Definition
Cicatricial pemphigoid, Bullous pemphigoid, erosive lichen planus, herpetic stomatitis, collapsed lesions may be white- leukoplakia |
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Term
Diagnosis of pemphigus vulgaris |
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Definition
Cytology, biopsy (histologic - intraepithelial vesicle formation, immunofluorescence - direct & indirect antibody levels correlate w/ disease activity) |
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Term
Direct immunofluorescence results of pemphigus vulgaris |
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Definition
Intercellular, IgG and C3 |
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Term
Indirect (circulating antibodies) results of pemphigus vulgaris |
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Definition
Present 80%, level correlates w/ disease activity |
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Term
Treatment of pemphigus vulgaris |
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Definition
Immune modulating medications, meticulous OH, control local factors intraorally |
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Term
Prognosis of pemphigus vulgaris |
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Definition
Noncurable, progressive disease; variable; corticosteroids and antibiotics are life-saving |
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Term
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Definition
- Uncommon
- Underlying lymphoma, thymoma, leukemia, Castleman's disease, etc.
- Clinically oral lesions resemble erythema multiforme or lupus erythematosus
- Oral lesions severe, large and painful, and often involve vermillion and perioral skin
- DIF: IgG to sqamous epi and all other desmosome-containing epi
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Term
Direct immunofluor results of paraneoplastic pemphigus |
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Definition
IgG intercellular, C3 BMZ, desmoplakin I, II, bullous pemphigoid antigen |
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Term
Indirect results for paraneoplastic pemphigus |
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Definition
polyvalent immunoglobulin to rat bladder transitional epithelium |
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Term
Prognosis of paraneoplastic pemphigus |
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Definition
Depends on control of underlying disease; due to benign tumor that can be surgically excised, good prognosis
Most have malignant neoplasms and die of disease or complications of treatment
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Term
Other names for progressive systemic sclerosis |
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Definition
Systemic sclerosis, scleroderma, hide bound disease |
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Term
Etiology of progressive systemic sclerosis |
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Definition
Unknown, prob autoimmune not contagious not inherited |
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Term
Diagnosis of progressive systemic sclerosis |
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Definition
Clinical and lab assessment (95% exhibit ANA) |
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Term
Clinical features of progressive systemic sclerosis |
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Definition
Thickened skin, mask-like facial features
Difficulty swallowing
Shortness of breath
Hand changes
Cold sensitivity in fingers Raynaud's phenomenon
Bone or joint pain
Generalized widening of PDL
Dry mouth, stenosis
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Term
Treatment of progressive systemic sclerosis |
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Definition
- Alleviate symptoms and minimize complications using corticosteroids and antimetabolites
- Hormone relaxin may reduce collagen overproduction
- Gleevac
- Dilate blood vessels, prevent blood clots
- Manage dry mouth
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Term
Prognosis of progressive systemic sclerosis |
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Definition
5 year survival rate 80-85%
Lung, heart, and kidney damage cause disability and death |
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Term
Other names for localized scleroderma |
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Definition
Morphea, circumscribed scleroderma, linear scleroderma "coupe de sabre" |
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