CH9 Allergy Part 2 Flashcards by Austin Shackelford

What systemic diseases can mimic orofacial granulomatosis

sarcoidosis
crohns
tuberculosis
chronic granulomatous disease

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What is the most common clinical presentation of orofacial granulomatosis

persistent painless lip swelling

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What are the three findings in Melkersson-Rosenthal syndrome?

Orofacial granulomatosis
Facial paralysis
Fissured tongue

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When only the lips are involved in orofacial granulomatosis what is the condition called?

cheilitis granulomatosa (of meischer)

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What type of granulomas are seen in orofacial granulomatosis

non-caseating granulomas around vessels

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How do you diagnose orofacial granulomatosis

process of elimination

last step is to try a cinnamon and benzoate free diet to see if it is an allergy

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Granulomatosis with polyangitis was previously called

Wegner’s granulomatosis

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What type of granulomas are seen in granulomatosis with polyangitis

necrotizing granulomas

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Demographics for wegners granulomatosis

wide range, average is 41
15% occur in under 20
90% Caucasians
3:`100,000 prevalence

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What are the 3 clinical patterns of Wegner and what are the criteria?

limited wegner granulomatosis = only respiratory involvement……generalized = upper/lower respiratory tract and renal involvment….superficial = lesions of the skin and mucosa

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What facial deformity can occur as a result of wegner granulomatosis

saddle nose deformity

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What is the most common cause of death in patients with granulomatosis with polyangitis

kidney involvement –> glomerulonephritis

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Oral involvement is the first sign of disease in _____% of people with wegners

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Characteristic ORAL clinical appearance of Wegners? Does it typically show before or after renal involvement? Which oral manifestation comes AFTER renal involvement?

strawberry gingivitis (early disease feature)
late disease is just non-specific ulceration...usually before renal involvement.....generalized ulcers come after renal involvement

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Histologic buzz words for granulomatosis with polyangitis

necrotizing granulomatous vasculitis
leukocytoclastic vasculitis
vessels with transmural inflammation
+/- subepithelial abscesses, pseudoepitheliomatous hyperplasia

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The american college of rheumatology diagnosis criteria (2 or more) for wegners granulomatosis

Oral ulcerations and nasal discharge
Nodules, fixed infiltrates, or cavities on chest radiograph
Abnormal urinary sediment (red blood cell casts or more than five red blood cells/high power field)
Granulomatous inflammation upon biopsy

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What serum test may be helpful in diagnosing wegners?

PR3-ANCA is the most helpful (formerly known as “c-ANCA”)….and less specific: MPO-ANCA (formerly “p-ANCA”)

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What % of patients have PR3-ANCA antibodies with granulomatosis with polyangitis

in 90-95% of patients of generalized patients

60% of localized/early cases

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Prognosis for wegners granulomatosis

Mean survival of generalized “classic” type = 5 months
80% by 1 year
90% by 2 years

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Relapse rate after 1+ years of treatment with prednisone and cyclophosphamide in wegners granulomatosis

30%

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% risk of adverse reaction with:
2 medications →
5 medications →
8+ drugs →

2 medications → 6% risk of adverse rxn
5 medications → 50% risk of adverse rxn
8+ drugs → nearly 100%

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What are the two broad categories of systemic drug reactions

Type A: augmented reactions

Type B: Bizarre reactions

Type A drug reactions are more frequent than type B accounting for ___%

80%

Type A definition

an expected but exaggerated effect

–too much bleeding on warfarin therapy

What is the most common Type B reaction

immune mediated hypersensitivity reaction

Patterns of stomatitis medicamentosa

1. fixed drug eruption 2. anaphylactic stomatitis 3. pemphigus-like/pemphigoid-like 4. lichenoid reaction 5. lupus erythematosus-like

What pattern is seen with indirect immunofluorescence of lichenoid drug reactions? (Ig?, pattern? antibody?)

IgG as a string of pearls along the basal cell layer of the strat squamous epithelium "basal cell cytoplasmic antibody"

Serum tests for lupus

ANAs | antibodies against dsDNA

How do you distinguish lupus erythematosus like drug reactions from true lupus?

Lupus-like drug reaction = circulating generic ANAs + antibodies against histones (NOT true for LLDR to TNF-a antagonists)...true lupus = Circulating generic ANAs + antibodies to histones + antibodies to dsDNA

____% of chronic cheilitis cases are a result of allergy

25%

Perioral dermatitis causes? Idiopatic, but possibly these 3? Also, what is the alternate/more appropriate name?

idiopathic, but related to uncritical use of potent corticosteroids on the facial skin, also toothpaste and heavy cosmetics implicated....PERIORIFICIAL dermatitis is more appropriate

primary demographic for periorificial dermatitis

Women 20-45 years old

What process? --> Persistent erythematous papules/pustules/vesicles around the mouth ___% include nasal area, ___% periorbital

Periorificial dermatitis 40% nasal 25% periorbital

Histologic findings in periorificial dermatitis...what can this be misdiagnosed as?

rosacea like pattern: Perifollicular granulomatous inflammation *also misdiagnosed as sarcoid

Contact stomatitis from artificial cinnamon resembles what condition that affects the gums

plasma cell gingivitis

What white lesion can contact stomatitis from artificial cinnamon resemble

oral hairy leukoplakia

Lichenoid contact reaction from dental materials is usually what type of reaction?

chronic type 4 hypersensitivity reactions

What component of amalgam typically causes lichenoid reaction? When are reactions to the amalgam filling more likely to occur - newly placed or old and corroded?

mercury...reactions more often occur with old, corroded restorations (you can have an acute rxn though, within hours of placement)

"Galvanic lesions"

older term for lichenoid reactions due to amalgam

Most commonly affected sites by lichenoid contact reactions

posterior buccal mucosa | lateral border of tongue

Patch testing is positive in up to ___% of patients with contact reactions vs only ___% of true LP patients

70% | 4%

Angioedema etiology (5) (mast cell degranulation, but why?)

1. IgE mediated hypersensitivity rxns 2. activation of the complement pathway (autoantibodies against C1-INH (hereditary or aquired) 3. ACE inhibitors (excess bradykinin) 4. high levels of antibody-antigen complexes: lupus, bacterial infections 5. Grossly elevated blood eosinophil counts

What drug can produce severe forms of angioedema? What race is most strongly associated with this?

ACE inhibitors...blacks (3-4x other races)

2 rare autosomal dominant hereditary conditions that lead to angioedema

both are a result of activation of the complement pathway Type 1: 85% of cases- quantitative reduction in C1-INH the inhibitor that prevents the transformation of C1 to C1 esterase Type 2: normal levels of CI-INH but it is dysfunctional

C1-INH deficiency caused by lymphoproliferative diseases

Caldwell syndrome

Another serum antibody (indirect immunofluorecense) sometimes tested for in wegner granulomatosis

MPO-ANCA myeloperoxidase, Typically not a finding in the oral cavity and is also associated with a wider variety of vasculopathies (formerly p-ANCA)

What are the two named clinical presentations of orofacial granulomatosis? (neither is specific lol)

1. Melkersson-Rosenthal syndrome 2. cheilitis granulomatosa (of Miescher)

Anaphylactic stomatitis arises after the allergen enters the circulation and binds to WHICH Ig?? + mast cell complexes

immunoglobulin E (IgE)

What is the alternate term for allergic contact stomatitis?

Stomatitis Venenata (latin for venom)

Gender predominance for acute and chronic allergic contact stomatitis?

distinct female

What is the most frequent symptom in acute contact stomatitis?

burning

How much time does it typically take for an acute contact stomatitis to manifest after exposure to the antigen?

within 30 minutes

What is the gold standard therapy for perioral dermatitis?

oral tetracycline

What are the 2 alternate names for angioedema?

1. AngioNeurotic Edema (old term) 2. Quincke Disease (the dude who first described it)