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Mucocutaneous Disease 2
Oral Pathology
40
Dentistry
Professional
10/16/2013

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Term
Alternative names for Peutz-Jeghers Syndrome
Definition

Hereditary intestinal

Polyposis Syndrome

Term
Etiology of Peutz-Jeghers Syndrome
Definition

Autosomal dominant, single pleotropic gene w/ high degree of penetrance LKB1 (encodes for serine/threonine kinase)

50% familial history, 35% spontaneous mutation

affects 1/120,000

Term
Clinical features of peutz-jeghers syndrome
Definition

Mucocutaneous pigmentation 90%: hairless melanotic macules on lips, buccal mucosa, gingiva, face, perianal and extremities

GI: multiple hamartomatous polyps

Respiratory system: may have polyps in nose and bronchioles

GU: benign ovarian sex-cord stromal tumors and polyps of bladder and ureter may occur

Term
Treatment & prognosis for peutz-jeghers syndrome
Definition

Manage complications: recurrent colicky abdominal pain, hemorrhage, obstruction and infarction due to intusussusception

Prognosis: GI carcinoma 2-3%; facial pigmentation may face, but oral persists

Term
Etiology of Epidermolysis Bullosa
Definition

(At least 25 diff types including:)

- Hereditary: Epidermolytic (EB simplex), Lamina lucidolytic (junctional), Dermolytic (dystrophic) - cutaneous squamous cell carcinoma, oral lesions most common

- Acquired epidermolysis bullosa

Term
Testing for hereditary epidermolysis bullosa
Definition

Direct immunofluorescence - No

Indirect (circulating antibodies) - No

-i.e. can't use either one for hereditary

Term
Testing for acquired epidermolysis bullosa
Definition

Direct - IgG (sub BMZ)

Indirect (circulating antibodies) - Yes

Term
Hereditary epidermolysis bullosa clinical features
Definition

Skin: blisters, scarring

Oral: ulcerations, blisters, microstomia

Dental: anodontia, enamel hypoplasia, neonatal teeth, dental caries

Term
Treatment and prognosis for epidermolysis bullosa
Definition

Symptomatic, avoid trauma, wound care; genetic counseling

Variable;cutaneous SCCA w/ recessive dystrophic forms

Term
Erythema migrans characteristics/etiology
Definition

-AKA Benign migratory glossitis or geographic tongue

Affects 1-3% of population

involves dorsum of tongue, although it can occur on any intraoral mucosal surface

More common females 2:1 males

Etiology: unknown possible hereditary and/or due to hypersensitivity to env factors

Term
Clinica features of erythema migrans
Definition

- Sensitivity to hot or spicy food

- Often occurs w/ fissured tongue

- May occur w/ migratory stomatitis - foci of erythema migrans on buccal mucosa, lips and/or soft palate

A small percentage of people experience constant burning or pain

- Occurs w/ increased freq in individuals w/ allergies and eczema

- Predisposing factors include psoriasis, inflammatory bowel diseas, anemia, allergies, and zinc deficiency

Term
If recent onset and painful erythema migrans:
Definition

May represent a hypersensitivity reaction (often to food or food additive)

It may be associated w/ onset of systemic disease (psoriasis, inflammatory bowel diseas, anemia, zinc deficiency - rare)

Term
Prognosis of erythema migrans
Definition

Excellent, completely benign condition

No treatment needed unless symptomatic

Chronic, non-curable condition

May be characterized by remission and exacerbation throughout life

Term
Definition of Erythema multiforme
Definition
Acute, inflammatory mucocutaneous disease
Term
Associations with erythema multiforme
Definition

30-70% associated with HSV

Association w/ HLA-B62 (same group that experiences HSV recurrences)

Term
Etiology of Erythema multiforme
Definition

Drugs, infections, idiopathic

Cinnamon

Recurrences triggered by HSV in 30-70% of cases; some cases Mycoplasma pneumonia

3m:1f

Adolescents and young adults

Term
Clinical features of erythema multiforme
Definition

Acute, inflammatory mucocutaneous disease, may be chronic

Very painful oral mucosal lesions

Crusted lips

Fever, joint pain

Skin-target lesions

Term
True or false: there are a lot of differential dxs of erythema multiforme
Definition
True
Term
Diagnosis of erythema multiforme
Definition
Clinical, biopsy (histologic - epidermal necrolysis, immunofluorescence), HSV1 and 2 antibody titers, HLA-B62
Term
Treatment for erythema multiforme
Definition
Glucocorticoids, CHX mouthrinse, avoid identifiable triggers, antiviral medications
Term
prognosis of erythema multiforme
Definition

Usually self-limiting, may last 4-6 weeks

May be recurrent

Rarely chronic

Rare variant: stevens-johnson syndrome (often requires hospitalization & may be life-threatening)

Term
Systemic lupus erythematosus
Definition

Autoimmune disease of unknown etiology (Viral infection? Sensitizing drugs such as hydralazine (antihypertensive))

Relatively common

Increased incidence in young and middle aged women

Diagnosis: clinical and laboratory findings

Term
Clinical features of SLE
Definition

- Discoid skin rash, scarring alopecia 80%

- Neurological disorder - cerebral lupus, immune complexes caught in blood vessels caus occlusion, seizures

- Malar skin rash - butterfly rash

- Skin rash: vasculitis

- Skin photosensitivity

- Oral ulcerations 20%

Term
How is renal disease caused from SLE?
Definition
Lupus nephritis due to collections of immune complexes in glomerular capillaries; may result in kidney failure
Term
Other characteristics of SLE
Definition

Serosal inflammation

Evidence of immunological disorder

Presence of antinuclear antibody (anti-dsDNA most freq detected; anti-ssDNA; anti-DNA-histone common in drug-induced SLE)

Normocytic hypochromic anemia, thrombocytopenia, hemolytic anemia, thrombophlebitis

Arthritis

Osteoporosis and avascular necrosis

Myalgia

Term
Treatment of SLE
Definition

Photosensitive-avoid sunlight

Corticosteroids and other immunosuppressive agents

Hydroxychloroquine (Plaquenil)

Term
Prognosis of SLE
Definition

95% 5 yr survival rate

75% 15 yr survival rate --> renal failure

Term
When diagnosis desquamative gingivitis
Definition

Requires biopsy w/ histologic diagnosis to establish diagnosis prior to treatment

May require immunohistochemical diagnosis

Most often diagnosed histologically

Term
Other names for cicatricial pemphigoid
Definition
Mucous membrane pemphigoid, benign mucous membrane pemphigoid
Term
Pathogenesis of cicatricial pemphigoid
Definition
Chronic autoimmune disease of unknown etiology; autoantigens involved in oral mucosal pemphigoid (BP antigen 1 and 2, Alpha-6 integrin subunit, epiligrin, laminin, 168-kd)
Term
Who does cicatricial pemphigoid effect?
Definition

82% female avg age 58

18% male avg age 62

Term
Oral involvement of cicatricial pemphigoid
Definition
Gingiva (92%), mucosa 18, tongue 7, pharynx 1, palate 17
Term
Extraoral involvement of cicatricial pemphigoid
Definition
Eye, skin, genitalia, nasal mucosa, esophagus
Term
Laboratory confirmation for cicatricial pemphigoid
Definition

Histopathology (formalin) 67% -- subepithelial split

Direct immunofluorescence 87% (Michel's solution) -- direct linear IgG and C3 at BMZ 80%; indirect uncommon

Term
Treatment of cicatricial pemphigoid
Definition
Immune modulating medications, meticulous oral hygiene, control local factors intraorally
Term
What does CREST stand for?
Definition
Calcinosis cutis, Raynaud's phenomenon, Esophageal dysfunction, sclerodactyly, telangiectasia
Term
Oral findings with CREST
Definition
Acquired microstomia, max interincisal opening < 40 mm, xerostomia (increased caries, DMF), tooth mobility, resorption of coronoid, condyle or angle of mandible, susceptibility to periodontitis, gingivits, recession, telangiectasia, trigeminal neuropathy, dysphagia, esophageal reflux
Term
Treatment of oral manifestations for CREST
Definition

Dry mouth: OH, CHX, sialogogues

Caries: Fl, diet, GI

Gingivitis: OH, CHX

Candidiasis

Microsomia: isometric exercises

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