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Mucocutaneous Disease 3
Oral pathology
46
Dentistry
Professional
10/24/2013

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Term
Another name for Hereditary Hemorrhagic Telangiectasia (HHT)
Definition
Osler-Weber-Rendu Syndrome
Term
Etiology of HHT
Definition
Rare, genetic disorder that affects blood vessels; autosomal dominant disorder; Affects 2-19/100,000
Term
HHT-1:
Definition
Mutation in a gene known as endoglin, located on chrom 9. Frequently associated w/ pulmonary and/or cerebra; arteriovenous malformations
Term
HHT-2
Definition
Due to gene on chrom 12 known as ALK1
Term
What are HHT-1 and HHT-2 associated w/?
Definition
Nosebleeds (epistaxis) and telangiectases
Term
Clinical features of HHT
Definition
Most dev telangiectasias (red spots formed by dilated blood vessels) often found on lips, tongue, fingers, or toes by age 30
Term
Treatment of HHT
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
Term
Prognosis of HHT
Definition
5% die from brain abscess exhibiting oral microflora
Term
Oral lichen planus
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


Term
Etiology of OLP
Definition
Idiopathic, multifactoral including drugs, dental materails, hep C, GVHD; immunopathogenesis involving T cells; immunologically mediated/autoimmune
Term
Clinical features of OLP
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
Term
Mucocutaneous clinical features of LP
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%

Term
Distribution of OLP (decreasing freq)
Definition
Buccal mucosa, tongue, gingiva, palate, floor of mouth, labial mucosa
Term
Management of OLP
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

Term
Differential diagnosis of OLP
Definition
Leukoplakia/epithelial dysplasia; desquamative gingival lesions; Lupus erythematosus; chronic ulcerative stomatitis
Term
OL Reactions
Definition
NSAIDs, Antihypertensive agents (angiotensin-converting enzyme inhibitors, Thiazides, diuretics, beta-blockers, calcium channel blockers), antimalarials, systemic gold
Term
Classification of OLP
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

Term
OLP management
Definition

Elimination of irritants/xerostomia

Modification of reversible lichenoid reactions (drugs, dental restorations)

Therapy (topical, systemic)

Term
Treatment of OLP
Definition

Topical therapy for biopsy confirmed painful or ulcerative disease

Corticosteroids, retinoids, tacrolimus, azathioprine, cyclosporine

Term
Therapy of OLP
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

Term
Malignant transformation of OLP
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

Term
Painful OLP
Definition

Consider removal of dental restoration(s) if lesions:

- Develop following placement

- Are located adj to restorations

Term
Summary of OLP
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

Term
Pathogenesis of Pemphigus Vulgaris
Definition
Circulating IgG autoantibodies to desmosomes of stratified squamous epithelium
Term
Definition of Pemphigus Vulgaris
Definition
Chronic autoimmune disease of unknown etiology; mucocutaneous; oral mucosa often precedes skin; usually middle aged adults
Term
Signs of Pemphigus vulgaris
Definition

Nikolsky's sign: minor trauma induces blister

Asboe-Hansen sign: blister will spread by pressing on it

Term
Clinical features of pemphigus vulgaris
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

Term
Intraoral sites of pemphigus vulgaris
Definition
Soft palate, buccal mucosa, tongue, gingiva
Term
True or false: Pemphigus vulgaris has variants.
Definition
True
Term
Differential diagnosis of pemphigus vulgaris
Definition
Cicatricial pemphigoid, Bullous pemphigoid, erosive lichen planus, herpetic stomatitis, collapsed lesions may be white- leukoplakia
Term
Diagnosis of pemphigus vulgaris
Definition
Cytology, biopsy (histologic - intraepithelial vesicle formation, immunofluorescence - direct & indirect antibody levels correlate w/ disease activity)
Term
Direct immunofluorescence results of pemphigus vulgaris
Definition
Intercellular, IgG and C3
Term
Indirect (circulating antibodies) results of pemphigus vulgaris
Definition
Present 80%, level correlates w/ disease activity
Term
Treatment of pemphigus vulgaris
Definition
Immune modulating medications, meticulous OH, control local factors intraorally
Term
Prognosis of pemphigus vulgaris
Definition
Noncurable, progressive disease; variable; corticosteroids and antibiotics are life-saving
Term
Paraneoplastic pemphigus
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

Term
Direct immunofluor results of paraneoplastic pemphigus
Definition
IgG intercellular, C3 BMZ, desmoplakin I, II, bullous pemphigoid antigen
Term
Indirect results for paraneoplastic pemphigus
Definition
polyvalent immunoglobulin to rat bladder transitional epithelium
Term
Prognosis of paraneoplastic pemphigus
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

Term
Other names for progressive systemic sclerosis
Definition
Systemic sclerosis, scleroderma, hide bound disease
Term
Etiology of progressive systemic sclerosis
Definition
Unknown, prob autoimmune not contagious not inherited
Term
Diagnosis of progressive systemic sclerosis
Definition
Clinical and lab assessment (95% exhibit ANA)
Term
Clinical features of progressive systemic sclerosis
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

Term
Treatment of progressive systemic sclerosis
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

Term
Prognosis of progressive systemic sclerosis
Definition

5 year survival rate 80-85%

Lung, heart, and kidney damage cause disability and death

Term
Other names for localized scleroderma
Definition
Morphea, circumscribed scleroderma, linear scleroderma "coupe de sabre"
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