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

Developmental and Immune-mediated Mucocutaneous Conditions - part II Flashcards

1
Q

mucous membrane pemphigoid (MMP) is also known as what?

A

cicatricial (scarring) pemphigoid

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2
Q

why does mucous membrane pemphigoid (MMP) resemble pemphigus vulgaris (PV)?

A

due to blister formation (i.e. pemphig”oid”)

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3
Q

T/F: pemphigus vulgaris (PV) is twice as common as mucous membrane pemphigoid (MMP)

A

false, MMP is twice as common as PV

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4
Q

what is the average age of pts dx’d with mucous membrane pemphigoid (MMP)?

A

50-60 y.o.

older age than PV

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5
Q

what is the gender predilection of mucous membrane pemphigoid (MMP)?

A

2:1 female predilection

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6
Q

where does mucous membrane pemphigoid (MMP) affect?

A

any mucosal surface, occasionally affects skin

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7
Q

scarring of mucous membrane pemphigoid (MMP) may appear where?

A
  1. skin

2. symblepheron (conjunctiva)

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8
Q

scarring on the oral mucosa due to mucous membrane pemphigoid (MMP) is common

A

false, rare

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9
Q

what might be seen in pts with mucous membrane pemphigoid (MMP) because the split is subepithelial?

A

intact intraoral blisters

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10
Q

clinical features of mucous membrane pemphigoid (MMP)

A

desquamative gingivitis

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11
Q

desquamative gingivitis

A

descriptive term: erythema, desquamation, ulceration

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12
Q

oral mucosal ulcers in pts with mucous membrane pemphigoid (MMP) are deeper than in pts with pemphigus vulgaris (PV)

A

true

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13
Q

what is the most significant aspect of mucous membrane pemphigoid (MMP)?

A

ocular involvement of sympblepheron

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14
Q

scarring of symblepheron in pts with mucous membrane pemphigoid (MMP) does what?

A

obstructs the orifices of glands that produces tears resulting in a dry eye

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15
Q

dry eye in pts with mucous membrane pemphigoid (MMP) leads to what?

A

keratinization of the corneal epithelium leading to blindness

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16
Q

entropion

A

upper eyelid turned inward

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17
Q

trichiasis

A

eyelashes rubbing against the eye

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18
Q

eyes of pts with mucous membrane pemphigoid (MMP)

A
  1. entropion
  2. trichiasis
  3. lower fornix is obliterated
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19
Q

biopsy of mucous membrane pemphigoid (MMP) must include what?

A
  1. generous sample of normal mucosa, 0.5-1 cm away from areas of ulceration/erythema, as epithelium easily strips off
  2. tissue should be submitted in both formalin and Michel’s solution
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20
Q

histo of mucous membrane pemphigoid (MMP)

A

SUBepithelial cleft formation

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21
Q

subepithelial cleft formation

A

separation of epithelium from CT at the basement membrane zone (BMZ)

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22
Q

immuno features of mucous membrane pemphigoid (MMP)

A
  1. linear deposition of immunoreactants at the BMZ

2. positive DIF; negative IIF

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23
Q

what percent of pts with mucous membrane pemphigoid (MMP) will have circulating autoantibodies?

A

only 5-25%

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24
Q

tx of mucous membrane pemphigoid (MMP) depends on what?

A

extent of involvement

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25
Q

tx of mucous membrane pemphigoid (MMP) oral lesions

A
  1. topic steroids - tetracycline/niacinamide or dapsone may be sufficient
  2. frequent dental prophylaxis, every 3-4 months
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26
Q

tx for mucous membrane pemphigoid (MMP)

A

refer pt to ophthalmologist for exam and f/u

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27
Q

what is indicated if there is ocular involvement in pts with mucous membrane pemphigoid (MMP)?

A

systemic imunnisuppressive therapy

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28
Q

T/F: px of mucous membrane pemphigoid (MMP) is rarely fatal

A

true

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29
Q

T/F: mucous membrane pemphigoid (MMP) can usually be controlled

A

true

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30
Q

blindness results in mucous membrane pemphigoid (MMP) pts with what?

A

with untreated ocular disease

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31
Q

T/F: mucous membrane pemphigoid (MMP) sometimes spontaneously resolve

A

false, rarely undergoes spontaneous resolution

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32
Q

which is the most common of autoimmune blistering conditions?

A

bullous pemphigoid (BP)

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33
Q

what is the average age of ppl who are affected by bullous pemphigoid (BP)?

A

older population, average age 75-80 y.o.

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34
Q

where does bullous pemphigoid (BP) primarily affect?

A

skin

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35
Q

T/F: bullous pemphigoid (BP) primarily appears on skin but can involve the mucous membrane as well

A

true

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36
Q

what is the gender predilection of bullous pemphigoid (BP)?

A

no gender predilection

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37
Q

what is an early symptom of bullous pemphigoid (BP)?

A

pruritus

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38
Q

what follows pruritus in bullous pemphigoid (BP)?

A

followed by development of multiple, tense bullae, blisters on normal or erythematous skin

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39
Q

T/F: lesions from bullous pemphigoid (BP) rupture, crust and heal with scarring

A

false, WITHOUT

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40
Q

T/F: oral involvement from bullous pemphigoid (BP) is common

A

false, uncommon

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41
Q

oral lesions of bullous pemphigoid (BP) rupture sooner than cutaneous lesions leaving what?

A

large shallow ulcerations with smooth, distinct margins

42
Q

histo features of bullous pemphigoid (BP)

A

SUBepithelial cleft similar to MMP

43
Q

immuno features of bullous pemphigoid (BP)

A

positive DIF AND IIF with immunoreactants deposited at the BMZ

44
Q

management of bullous pemphigoid (BP) is similar to cicatricial pemphigoid but most BP cases what?

A

resolve spontaneously in 1-2 years

45
Q

T/F: most bullous pemphigoid (BP) pts experience remission

A

true

46
Q

why is mortality of bullous pemphigoid (BP) pts 3x higher than age and sex matched population?

A

problems may develop with use of immunosuppresive therapy in older populations

47
Q

T/F: etiology of erythema multiforme (EM) is probably immune-mediated

A

true

48
Q

immune-mediated condition

A

immunologic derangement stimulated by trigger that produces that disease

49
Q

etiology break down of erythema multiforme (EM)

A

50% - unknown
25% - preceding infection
25% medication-related

50
Q

25% of erythema multiforme (EM) cases are due to what type of preceding infections?

A
  1. viral (herpes)

2. bacterial (mycoplasma pneumoniae)

51
Q

25% of erythema multiforme (EM) cases are related to what type of medications?

A
  1. abx

2. analgesics

52
Q

who is affected by erythema multiforme (EM)?

A

young adult, 20-30’s

53
Q

clinical features of erythema multiforme (EM)

A
  1. acute onset ulcerative disorder skin and mucous membranes
  2. prodromal symptoms ~1 wk before onset
  3. varying degrees of severity
54
Q

prodromal symptoms of erythema multiforme (EM)

A
  1. fever
  2. malaise
  3. headache
  4. cough
  5. sore throat
55
Q

previous studies showed that erythema multiforme (EM) has a male predilection but more recent studies show what?

A

a female predilection

56
Q

how long do pts experience prodromal symptoms before onset of erythema multiforme (EM)?

A

~1 week

57
Q

varying degrees of severity of erythema multiforme (EM)

A
  1. EM minor

2. EM major

58
Q

EM minor of erythema multiforme (EM) MUCOSA features

A
  1. hemorrhagic crusting of vermilion zones
  2. erythematous patches oral mucosa that undergo necrosis and result in large, shallow erosions and ulcers with irregular borders
59
Q

which locations are affected by EM minor of erythema multiforme (EM)?

A
  1. skin (extremities)

2. mucosa (oral, conjunctival, genitourinary, respiratory)

60
Q

what mucosa in the oral cavity is usually spared from EM minor of erythema multiforme (EM)?

A
  1. gingiva

2. hard palate

61
Q

EM minor of erythema multiforme (EM) SKIN features

A
  1. variety of appearances “multiforme”
  2. round, dusky-red patches on skin of extremities “target lesions”
  3. bullae with necrotic centers
62
Q

EM major of erythema multiforme (EM) clinical features

A
  1. 2 or more mucosal sites in conjunction with skin lesions

2. ocular involvement can produce symblepheron

63
Q

which sites are affected by EM major of erythema multiforme (EM) AND EM minor?

A
  1. mucosal
  2. lip
  3. skin
64
Q

different processes of erythema multiforme (EM) than EM major and minor

A
  1. Stevens-Johnson syndrome (SJS)

2. toxic epidermal necrolysis (TEN)

65
Q

Stevens-Johnson syndrome (SJS)

A

at least two mucosal sites plus skin involvement

66
Q

toxic epidermal necrolysis (TEN) is also known as what?

A

Lyell’s disease

67
Q

toxic epidermal necrolysis (TEN)

A

diffuse bullous involvement of skin and mucosa

68
Q

what is the difference between SJS and TEN?

A

degree of skin involvement and age

69
Q

SJS has what percent of skin involvement and usually affects who compared to TEN?

A

<10% of skin invovlement and usually younger pt

70
Q

TEN has what percent of skin involvement and usually affect who compared to SJS?

A

> 30% skin involvement and usually over 60 y.o.

71
Q

distinguishing features between EM and SJS/TEN

A
  1. SJS and TEN almost always triggered by a drug, rather than an infection
  2. SJS and TEN skin lesions begin as red macules or trunk rather than extremities
72
Q

clinical features of SJS and TEN

A
  1. within 2 weeks skin sloughing and flaccid bullae develop

2. pts may appear badly scalded - usually tx’d in burn unit

73
Q

almost all SJS and TEN pts have what?

A

mucosal involvement esp oral

74
Q

dx of EM, SJS and TEN are usually based on what?

A

clinical presentation

75
Q

T/F: DIF and IIF are non-specific so it’s not helpful except in ruling out other immune-mediated conditions

A

true

76
Q

histo features of EM, SJS, TEN

A
  1. subepithelial or intraepithelial vesicles
  2. necrotic basal keratinocytes
  3. mixed inflammatory cell infiltrate
  4. may see perivascular inflammation
77
Q

supportive therapy for erythema multiforme (EM)

A
  1. discontinue causative drug!
  2. systemic or topical steroids early on
  3. IV re-hydration
  4. topic anesthetic or analgesic for pain
78
Q

tx of SJS and TEN

A
  1. discontinue causative drug!
  2. IV re-hydration
  3. topical anesthetic or analgesic for pain
79
Q

what has been implicated in inducing TEN?

A

NSAIDS

80
Q

why should steroids be avoided in managing TEN?

A

associated with increased mortality

81
Q

how does IV administration of pooled human immunoglobulins seems helpful in resolution of TEN?

A

by blocking apoptosis of epithelial cells

82
Q

EM minor and major are self-limiting and should resolve in how many weeks?

A

2-6 wks

83
Q

20% of EM minor and major pts get recurrences especially during what time(s)?

A

spring and autumn

84
Q

if HSV triggered EM minor or major, what is indicated for maintenance?

A

antiviral

85
Q

T/F: erythema multiforme (EM) is not life-threatening except when?

A

severe cases

86
Q

mortality rate of SJS

A

1-5%

87
Q

mortality rate of TEN

A

25-30%

88
Q

px of pts that survives from SJS or TEN

A
  1. skin heals in 3-5 wks
  2. oral lesions take longer
  3. ocular damage in 50% of pts
89
Q

erythema migrans is also known as what?

A
  1. geographic tongue

2. benign migratory glossitis

90
Q

etiopathogenesis of erythema migrans is unknown but is probably what?

A

immune-mediated

91
Q

erythema migrans is one ofhte more common oral conditions occurring in what percent of the population?

A

1-3% so considered “common oral lesion”

92
Q

erythema seen in erythema migrans is due to what?

A

atrophy of filiform papillae and shearing off of the parakeratin causing epithelium to thin resulting in red appearance

93
Q

erythema migrans occurs in 1/3rd of pts who have what?

A

fissured tongue

94
Q

clinical features of erythema migrans

A
  1. wax and wane
  2. heal then develop in different area
  3. multiple, well-demarcated zones of erythema surrounded at least partially by slightly elevated yellow-white serpentine or scalloped border
  4. can be singular “persistens”
95
Q

where does erythema migrans characteristically affect on the tongue?

A

dorsal and lateral anterior 2/3 of tongue

96
Q

ectopic geographic tongue

A

erythema migrans on other non-keratinized mucosal surfaces

97
Q

histo of erythema migrans is similar to what?

A

psoriasis “psoriasiform mucositis” description on bx report

98
Q

histo of erythema migrans

A
  1. parakeratosis with extensive microabscess (neutrophils) formation in superficial spinous layer
  2. elongation of rete ridges
  3. neutrophilic infiltration
99
Q

tx of erythema migrans

A

none

100
Q

occassionaly pts with erythema migrans are sensitive or painful to what?

A

hot, spicy foods… can use potent topical steroid

101
Q

px of erythema migrans

A

good

102
Q

T/F: pts with erythema migrans should be reassured that this is a benign condition

A

true

AU'18 Oral Path II (31 decks)

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