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oral path 2 final > Immune-Mediated Mucocutaneous disease > Flashcards

Immune-Mediated Mucocutaneous disease Flashcards

1
Q

Pemphigus Vulgaris is a disease characterized by autoantibodies that destroy ____________

A

desmosomes

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2
Q

what population groups are most likely to have pemphigus Vulgaris?

A
  • Adults, 4th to 6th decades
  • no sex predilection
  • Relatively rare
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3
Q

T/F: Pemphigus vulgaris is usually fatal if not treated

A

True

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4
Q

over ___% of patients with pemphigus vulgaris will present with oral symptoms

A

50%

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5
Q

what are the clinical characteristics of pemphigus vulgaris?

A

Ragged erosions and ulcerations

Any oral mucosal surface

Flaccid bullae on skin; intact oral blisters rarely seen

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6
Q

T/F: Oral lesions of pemphigus are often the initial manifestation of the disease and are the first symptoms to resolve after therapy has begun

A

FALSE

Oral lesions are often the initial manifestation of the disease and the most difficult to resolve with therapy

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7
Q

The “_________ sign” is a clinical hallmark of Pemphigus vulgaris

A

Nikolsky sign

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8
Q

what do antibodies bind to during Pemphigus vulgaris?

A

Autoantibodies bind desmosomal components (desmoglein 3 &1)

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9
Q

T/F: during testing for Pemphigus Vulgaris, Both direct (DIF) and indirect (IIF) immunofluorescence studies will be positive

A

true

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10
Q

what immunoglobulin is used during both indirect and direct immunofluorescence studies?

A

Fluorescein-labeled, anti-human IgG

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11
Q

what are the histopathological findings of Pemphigus Vulgaris?

A

1) Intraepithelial (aka suprabasilar) clefting
2) Acantholysis (breakdown of spinous layer) – is also usually evident
3) “Dilapidated brick wall” with INTACT BASAL CELL LAYER

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12
Q

what treatments are used for pemphigus vulgaris?

A

1) Systemic corticosteroids, often with azathioprine or other steroid-sparing agents
2) Topical corticosteroids have little effect

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13
Q

what is the mortality rate for Pemphigus vulgaris? what was it before the use of systemic corticosteroids?

A

Prior to corticosteroid therapy, 60-90% mortality

Today, 5-10% mortality, usually due to complications of therapy

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14
Q

For Pemphigus vulgaris, what is the saying that pertains to the oral lesions?

A

“First to show and last to go.”

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15
Q

“Mucous Membrane Pemphigoid” is also known by what other name?

A

Also known as cicatricial pemphigoid

cicatricial means “scarring”

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16
Q

MMP will clinically resemble ________ due to blister formation

A

pemphigus

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17
Q

what population groups are most likely to have Mucous membrane Pemphigoid (MMP)?

A

A) 2:1 female predilection

B) Older age group – average age, 50-60 years

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18
Q

T/F: Pemphigus Vulgaris is more common than Mucous Membrane Pemphigoid

A

FALSE

MMP is 2-4 X more common than pemphigus

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19
Q

when is scarring usually seen in MM Pemphigoid?

A

Scarring usually seen with conjunctival (symblepharon) and cutaneous lesions

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20
Q

What are the clinical characteristics of MM Pemphigoid?

A

A) May affect any mucosal surface; occasionally skin

B) Often presents as desquamative gingivitis

C) May see intact blisters intraorally

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21
Q

T/F: Scarring from MM pemphigoid is rare intraorally, but is more common with skin (cutaneous) involvement

A

true

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22
Q

What is the most significant aspect of MM pemphigoids?

A

Ocular scarring

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23
Q

what causes ocular scarring from MM Pemphigoid?

A

1) Scarring obstructs the orifices of glands that produce the tear film, resulting in a dry eye
2) Dryness leads to keratinization of the corneal epithelium, leading to blindness
3) Scarring may lead to adhesion formation (symblepharons) between eyelid and globe

24
Q

list the histopathological characteristics of MM pemphigoid:

A

Microscopic examination shows:
A) clean subepithelial cleft formation

B) separation of the intact epithelium from the connective tissue at the BMZ

25
Q

which immunofluorescence study would be positive during MM Pemphigoid? which would be negative?

A

Positive DIF

IIF usually negative

26
Q

what epithelial layer is effected during MM Pemphigoid

A
  • the basement membrane zone

- Linear deposition of immunoreactants at the BMZ

27
Q

Pemphigus effects the _________ of epithelial cells, while Pemphigoids effect the __________

A

Pemphigus = Desmosomes

Pemphigoids = Hemidesmosomes & BMZ

28
Q

what are the treatments for MM Pemphigoid?

A

A) Oral lesions only - topical steroids (first line), tetracycline/niacinamide or dapsone may be sufficient

B) If ocular involvement is present, systemic immunosuppressive therapy is indicated

29
Q

what is the prognosis for MM Pemphigoid?

A

1) Rarely fatal
2) Condition can usually be controlled
3) Blindness results in patients with untreated ocular disease
4) Rarely undergoes spontaneous resolution

30
Q

in what population groups are Bullous Pemphigoid most commonly found?

A

Usually older adults, 75-80 yrs

31
Q

T/F: oral involvement is common in Bullous Pemphigoid

A

FALSE

only 10-20% will have oral involvement

32
Q

what are the initial symptoms of Bullous Pemphigoid?

A

Pruritus (itching) is common initial complaint, followed by cutaneous blisters

33
Q

how do Bullous Pemphigoid and MM Pemphigoid differ when doing a immunofluorescence study?

A

Both will have Subepithelial clefts

A) For Bullous Pemphigoid- Positive DIF and IIF

B) For MM pemphigoid- only the DIF is positive

34
Q

T/F: Like MM Pemphigoid, Bullous Pemphigoid effects the basement membrane zone of epithelial cells

A

True

35
Q

how is Bullous Pemphigoid treated?

A

Management similar to MM pemphigoid, but immunosuppressive therapy can have serious side effects

Many cases resolve spontaneously in 1-2 years

36
Q

what histopath characteristic is seen in both Bullous and MM Pemphigoid?

A

Subepithelial clefts

37
Q

_______________ is an ACUTE, self-limiting ulcerative disorder

A

Erythema Multiforme

38
Q

what is the Etiology of Erythema Multiforme?

A
  • 50% - unknown

- 50% - most are infection-related (often HSV) or (less commonly) medication-related

39
Q

who is most likely to acquire Erythema Multiforme?

A

Young adult females

40
Q

what are the 3 forms of Erythema Multiforme?

A

A) EM minor

B) EM major

C) Stevens-Johnson syndrome & toxic epidermal necrolysis

41
Q

how does EM minor differ from EM major?

A

EM minor – skin or oral mucosa only

EM major – at least TWO mucosal sites PLUS skin involvement

42
Q

what is the difference between Stevens-Johnson syndrome & toxic epidermal necrolysis?

(reminder, they are both forms of Erythema Multiforme)

A

Stevens-Johnson: less than 10% of body involved

TEN: over 30% of body is involved

43
Q

What are the Clinical characteristics of Erythema Multiforme?

A

** A) Hemorrhagic crusting of lips **

B) Widespread oral ulcers with ragged margins

C) “target” lesions of skin

44
Q

what oral sites are commonly effected during Erythema Multiforme?

A

Labial mucosa, buccal mucosa and tongue

45
Q

T/F: the DIF results for Erythema Multiforme will be non-specific

A

True

46
Q

how is Erythema Multiforme treated?

give treatment for EM major, minor, and Toxic epidermal necrolysis

A

1) Supportive care (analgesics, soft diet, hydration) for mild cases
2) Somewhat controversial for EM major – corticosteroids are often given empirically
3) TEN managed in burn unit; IV pooled human immunoglobulin shows promise

47
Q

what is the PROGNOSIS for Erythema Multiforme?

give prognosis for EM major, minor, and Toxic epidermal necrolysis

A

Good for mild to moderate cases

EM major – 1-5% mortality

TEN – 25-30% historically; IV Ig therapy has dramatically improved patient recovery

48
Q

what is the recurrence rate for Erythema Multiforme?

WHEN is recurrence most likely?

A

EM may be recurrent in 20% of cases

– usually autumn & spring

49
Q

“benign migratory glossitis” is a form of what disease?

A

Erythema migrans

50
Q

T/F: Benign Migratory Glossitis is one of the most common oral conditions

A

True

One of the more common oral conditions, occurring in 1-3% of the population

51
Q

benign migratory glossitis (erythema migrans) is also known as “______________”

A

geographic tongue

52
Q

what are the clinical characteristics of benign migratory glossitis (erythema migrans)?

A

A) Often seen with fissured tongue

B) Patients typically report waxing and waning of lesions

C) May develop on other non-keratinized mucosal surfaces

53
Q

what is the term given for benign migratory glossitis developing on NON-keratinized mucosal surfaces other than the tongue?

A

“ectopic geographic tongue”

54
Q

T/F: the histopathology for benign migratory glossitis is similar to psoriasis

A

true

biopsies are often diagnosed as “psoriasiform mucositis”

55
Q

what are the histopath characteristics of benign migratory glossitis?

A

Parakeratosis with extensive microabscess formation in the UPPER SPINOUS layer

  • Results in LOSS of SUPERFICIAL PARAKERATIN
  • Remaining epithelium is much thinner and appears red
56
Q

what is the treatment protocol for Benign Migratory Glossitis?

A

A) No treatment is generally necessary

B) Some patients may complain of sensitivity to hot or spicy foods when lesions are active

C) May respond to one of the stronger topical corticosteroid gel preparations

57
Q

what are the microscopic characteristics of Erythema Multiforme lesions?

A

1) Keratinocyte destruction
2) sub epithelial edema
3) mixed inflammatory infiltrate
4) perivascular inflammation

oral path 2 final (3 decks)

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