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AU'18 Oral Path II > Developmental and Immune-mediated Mucocutaneous Conditions - part I COPY > Flashcards

Developmental and Immune-mediated Mucocutaneous Conditions - part I COPY Flashcards

1
Q

categories of mucocutaneous conditions

A
  1. developmental

2. immune-mediated

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2
Q

developmental mucocutaneous conditions

A
  1. ectodermal dysplasia
  2. white sponge nevus
  3. Peutz-Jeghers syndrome
  4. Hereditary hemorrhagic telangiectasia
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3
Q

immune-mediated mucocutaneous conditions

A
  1. pemphigus vulgaris
  2. mucous membrane pemphigoid
  3. bullous pemphigoid
  4. erythema multiforme
  5. erythema migrans (geographic tongue)
  6. linchen planus
  7. lichenoid mucositis
  8. lupus erthematosus
  9. systemic sclerosis
  10. CREST syndrome
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4
Q

etiology of pemphigus vulgaris (PV)

A

autoimmune

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5
Q

Pemphigus vulgaris (PV) is due to inappropriate production of what?

A

antibodies by the host directed against host tissue (autoantibodies)…

damage to host by host’s own immune response

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6
Q

Pemphigus vulgaris (PV) autoantibodies destroy what?

A

desmosomes

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7
Q

role of desmosomes

A

bond epithelial cells together

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8
Q

what happens when antibodies inhibit desmosomes?

A

inhibits adherence so a split develops in the epithelium

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9
Q

T/F: Pemphigus vulgaris (PV) is relatively rare

A

true, ~5 cases per million dx’d each year in general population

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10
Q

what is the average age of pts dx’d with pemphigus vulgaris (PV)?

A

50 y.o.

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11
Q

what is the gender predilection for pemphigus vulgaris (PV)?

A

no gender predilection

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12
Q

oral lesions in pts with pemphigus vulgaris (PV)

A
  1. superficial, ragged erosions and ulcerations

2. any mucosal surface

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13
Q

T/F: the oral lesions in pts with pemphigus vulgaris (PV) are the “first to show, last to go”

A

true, oral lesions are the initial manifestation of the disease and the most difficult to resolve with treatment

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14
Q

clinical features of pemphigus vulgaris (PV)

A
  1. flaccid vesicles, bullae on skin

2. + Nikolsky sign

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15
Q

+ Nikolsky sign

A

inducing a bulla by applying firm, lateral pressure to normal appearing skin

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16
Q

what is rarely seen intact intraorally in pts with pemphigus vulgaris (PV)?

A

flaccid vesicles, bullae

17
Q

what is important in managing pemphigus vulgaris (PV)?

A

plaque control

18
Q

normal tissue adjacent to ulceration or erosion of pemphigus vulgaris (PV) should be sampled for what?

A
  1. direct immunoflorescent (in Michel’s solution)

2. light microscopic evaluation

19
Q

histo of pemphigus vulgaris (PV)

A
  1. INTRAEPITHELIAL clefting above the basal layer (i.e. within the epithelium)
  2. acantholysis
20
Q

acantholysis

A

breakdown of spinous layer; cells appear to fall apart

21
Q

what remains attached to the CT seen microscopically in pemphigus vulgaris (PV)?

A

only basal cell layer of epithelium

22
Q

immunopathology identifies what?

A

immune responses associated with diseases

23
Q

immunofluorescence

A

technique that uses fluorescent-labeled antibodies to detect specific targets

24
Q

T/F: immunopathology is used to help identify several immune-mediated conditions

A

true

25
Q

direct immunofluorescence (DIF) is used to detect what?

A

autoantibodies bound to pt’s tissues

26
Q

indirect immunofluorescence (IIF) is used to detect what?

A

antibodies circulating in the blood

27
Q

immuno features of pemphigus vulgaris (PV)

A
  1. both DIF and IIF studies will be positive in pemphigus vulgaris
  2. autoantibodies bind desmosomal components (desmoglein 1 & 3)
28
Q

DIF-immunoreactants are what in pemphigus vulgaris (PV)?

A

in intercellular areas b/w the surface epithelial cells “chicken wire” with SUPRAbasilar cleft

29
Q

tx of pemphigus vulgaris (PV)

A

systemic corticosteroids - often azathiprine or other steroid-sparing agents

30
Q

why does topical corticosteroids have little effect in tx’ing pemphigus vulgaris (PV)?

A

PV is a systemic disease

31
Q

what percent of pemphigus vulgaris (PV) cases will resolve on their own after 10 years?

A

~30%

32
Q

T/F: pemphigus vulgaris (PV) is usually fatal if not tx’d

A

true

33
Q

why may pemphigus vulgaris (PV) be fatal if not tx’d?

A
  1. severe infection
  2. loss of fluids/electrolytes
  3. malnutrition due to mouth pain
34
Q

prior to corticosteroid therapy, what mortality rates of pemphigus vulgaris (PV) cases were what percent?

A

60-90%

35
Q

what may lead to mortality of pts with pemphigus vulgaris (PV)?

A

complications of long-term steroid

36
Q

what is the morality rate of pts with pemphigus vulgaris (PV) today?

A

5-10% mortality

37
Q

mortality today in pts with pemphigus vulgaris (PV) is usually due to what?

A

complications of therapy (side effect of steroids, immune-suppression)

38
Q

what drug is used to tx pemphigus vulgaris (PV) suppresses bone marrow and is a carcinogen?

A

azathioprine

AU'18 Oral Path II (31 decks)

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