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Neville and Damm > CH9 Allergy Part 2 > Flashcards

CH9 Allergy Part 2 Flashcards

1
Q

What systemic diseases can mimic orofacial granulomatosis

A

sarcoidosis
crohns
tuberculosis
chronic granulomatous disease

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2
Q

What is the most common clinical presentation of orofacial granulomatosis

A

persistent painless lip swelling

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3
Q

What are the three findings in Melkersson-Rosenthal syndrome?

A

Orofacial granulomatosis
Facial paralysis
Fissured tongue

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4
Q

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

A

cheilitis granulomatosa (of meischer)

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5
Q

What type of granulomas are seen in orofacial granulomatosis

A

non-caseating granulomas around vessels

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6
Q

How do you diagnose orofacial granulomatosis

A

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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7
Q

Granulomatosis with polyangitis was previously called

A

Wegner’s granulomatosis

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8
Q

What type of granulomas are seen in granulomatosis with polyangitis

A

necrotizing granulomas

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9
Q

Demographics for wegners granulomatosis

A

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

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10
Q

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

A

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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11
Q

What facial deformity can occur as a result of wegner granulomatosis

A

saddle nose deformity

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12
Q

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

A

kidney involvement –> glomerulonephritis

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13
Q

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

A

2%

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14
Q

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

A 
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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15
Q

Histologic buzz words for granulomatosis with polyangitis

A

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

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16
Q

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

A
  • 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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17
Q

What serum test may be helpful in diagnosing wegners?

A

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

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18
Q

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

A

in 90-95% of patients of generalized patients

60% of localized/early cases

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19
Q

Prognosis for wegners granulomatosis

A

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

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20
Q

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

A

30%

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21
Q

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

A

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

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22
Q

What are the two broad categories of systemic drug reactions

A

Type A: augmented reactions

Type B: Bizarre reactions

23
Q

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

A

80%

24
Q

Type A definition

A

an expected but exaggerated effect

–too much bleeding on warfarin therapy

25
Q

What is the most common Type B reaction

A

immune mediated hypersensitivity reaction

26
Q

Patterns of stomatitis medicamentosa

A
  1. fixed drug eruption
  2. anaphylactic stomatitis
  3. pemphigus-like/pemphigoid-like
  4. lichenoid reaction
  5. lupus erythematosus-like
27
Q

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

A

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

28
Q

Serum tests for lupus

A

ANAs

antibodies against dsDNA

29
Q

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

A

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

30
Q

____% of chronic cheilitis cases are a result of allergy

A

25%

31
Q

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

A

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

32
Q

primary demographic for periorificial dermatitis

A

Women 20-45 years old

33
Q

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

A

Periorificial dermatitis
40% nasal
25% periorbital

34
Q

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

A

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

35
Q

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

A

plasma cell gingivitis

36
Q

What white lesion can contact stomatitis from artificial cinnamon resemble

A

oral hairy leukoplakia

37
Q

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

A

chronic type 4 hypersensitivity reactions

38
Q

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?

A

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

39
Q

“Galvanic lesions”

A

older term for lichenoid reactions due to amalgam

40
Q

Most commonly affected sites by lichenoid contact reactions

A

posterior buccal mucosa

lateral border of tongue

41
Q

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

A

70%

4%

42
Q

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

A
  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
43
Q

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

A

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

44
Q

2 rare autosomal dominant hereditary conditions that lead to angioedema

A

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

45
Q

C1-INH deficiency caused by lymphoproliferative diseases

A

Caldwell syndrome

46
Q

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

A

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

47
Q

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

A
  1. Melkersson-Rosenthal syndrome 2. cheilitis granulomatosa (of Miescher)
48
Q

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

A

immunoglobulin E (IgE)

49
Q

What is the alternate term for allergic contact stomatitis?

A

Stomatitis Venenata (latin for venom)

50
Q

Gender predominance for acute and chronic allergic contact stomatitis?

A

distinct female

51
Q

What is the most frequent symptom in acute contact stomatitis?

A

burning

52
Q

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

A

within 30 minutes

53
Q

What is the gold standard therapy for perioral dermatitis?

A

oral tetracycline

54
Q

What are the 2 alternate names for angioedema?

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

Neville and Damm (72 decks)

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