Developmental and Immune-mediated Mucocutaneous Conditions - part II Flashcards
(102 cards)
1
Q
mucous membrane pemphigoid (MMP) is also known as what?
A
cicatricial (scarring) pemphigoid
2
Q
why does mucous membrane pemphigoid (MMP) resemble pemphigus vulgaris (PV)?
A
due to blister formation (i.e. pemphig”oid”)
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
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
5
Q
what is the gender predilection of mucous membrane pemphigoid (MMP)?
A
2:1 female predilection
6
Q
where does mucous membrane pemphigoid (MMP) affect?
A
any mucosal surface, occasionally affects skin
7
Q
scarring of mucous membrane pemphigoid (MMP) may appear where?
A
- skin
2. symblepheron (conjunctiva)
8
Q
scarring on the oral mucosa due to mucous membrane pemphigoid (MMP) is common
A
false, rare
9
Q
what might be seen in pts with mucous membrane pemphigoid (MMP) because the split is subepithelial?
A
intact intraoral blisters
10
Q
clinical features of mucous membrane pemphigoid (MMP)
A
desquamative gingivitis
11
Q
desquamative gingivitis
A
descriptive term: erythema, desquamation, ulceration
12
Q
oral mucosal ulcers in pts with mucous membrane pemphigoid (MMP) are deeper than in pts with pemphigus vulgaris (PV)
A
true
13
Q
what is the most significant aspect of mucous membrane pemphigoid (MMP)?
A
ocular involvement of sympblepheron
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
15
Q
dry eye in pts with mucous membrane pemphigoid (MMP) leads to what?
A
keratinization of the corneal epithelium leading to blindness
16
Q
entropion
A
upper eyelid turned inward
17
Q
trichiasis
A
eyelashes rubbing against the eye
18
Q
eyes of pts with mucous membrane pemphigoid (MMP)
A
- entropion
- trichiasis
- lower fornix is obliterated
19
Q
biopsy of mucous membrane pemphigoid (MMP) must include what?
A
- generous sample of normal mucosa, 0.5-1 cm away from areas of ulceration/erythema, as epithelium easily strips off
- tissue should be submitted in both formalin and Michel’s solution
20
Q
histo of mucous membrane pemphigoid (MMP)
A
SUBepithelial cleft formation
21
Q
subepithelial cleft formation
A
separation of epithelium from CT at the basement membrane zone (BMZ)
22
Q
immuno features of mucous membrane pemphigoid (MMP)
A
- linear deposition of immunoreactants at the BMZ
2. positive DIF; negative IIF
23
Q
what percent of pts with mucous membrane pemphigoid (MMP) will have circulating autoantibodies?
A
only 5-25%
24
Q
tx of mucous membrane pemphigoid (MMP) depends on what?
A
extent of involvement
25
tx of mucous membrane pemphigoid (MMP) oral lesions
1. topic steroids - tetracycline/niacinamide or dapsone may be sufficient
2. frequent dental prophylaxis, every 3-4 months
26
tx for mucous membrane pemphigoid (MMP)
refer pt to ophthalmologist for exam and f/u
27
what is indicated if there is ocular involvement in pts with mucous membrane pemphigoid (MMP)?
systemic imunnisuppressive therapy
28
T/F: px of mucous membrane pemphigoid (MMP) is rarely fatal
true
29
T/F: mucous membrane pemphigoid (MMP) can usually be controlled
true
30
blindness results in mucous membrane pemphigoid (MMP) pts with what?
with untreated ocular disease
31
T/F: mucous membrane pemphigoid (MMP) sometimes spontaneously resolve
false, rarely undergoes spontaneous resolution
32
which is the most common of autoimmune blistering conditions?
bullous pemphigoid (BP)
33
what is the average age of ppl who are affected by bullous pemphigoid (BP)?
older population, average age 75-80 y.o.
34
where does bullous pemphigoid (BP) primarily affect?
skin
35
T/F: bullous pemphigoid (BP) primarily appears on skin but can involve the mucous membrane as well
true
36
what is the gender predilection of bullous pemphigoid (BP)?
no gender predilection
37
what is an early symptom of bullous pemphigoid (BP)?
pruritus
38
what follows pruritus in bullous pemphigoid (BP)?
followed by development of multiple, tense bullae, blisters on normal or erythematous skin
39
T/F: lesions from bullous pemphigoid (BP) rupture, crust and heal with scarring
false, WITHOUT
40
T/F: oral involvement from bullous pemphigoid (BP) is common
false, uncommon
41
oral lesions of bullous pemphigoid (BP) rupture sooner than cutaneous lesions leaving what?
large shallow ulcerations with smooth, distinct margins
42
histo features of bullous pemphigoid (BP)
SUBepithelial cleft similar to MMP
43
immuno features of bullous pemphigoid (BP)
positive DIF AND IIF with immunoreactants deposited at the BMZ
44
management of bullous pemphigoid (BP) is similar to cicatricial pemphigoid but most BP cases what?
resolve spontaneously in 1-2 years
45
T/F: most bullous pemphigoid (BP) pts experience remission
true
46
why is mortality of bullous pemphigoid (BP) pts 3x higher than age and sex matched population?
problems may develop with use of immunosuppresive therapy in older populations
47
T/F: etiology of erythema multiforme (EM) is probably immune-mediated
true
48
immune-mediated condition
immunologic derangement stimulated by trigger that produces that disease
49
etiology break down of erythema multiforme (EM)
50% - unknown
25% - preceding infection
25% medication-related
50
25% of erythema multiforme (EM) cases are due to what type of preceding infections?
1. viral (herpes)
| 2. bacterial (mycoplasma pneumoniae)
51
25% of erythema multiforme (EM) cases are related to what type of medications?
1. abx
| 2. analgesics
52
who is affected by erythema multiforme (EM)?
young adult, 20-30's
53
clinical features of erythema multiforme (EM)
1. acute onset ulcerative disorder skin and mucous membranes
2. prodromal symptoms ~1 wk before onset
3. varying degrees of severity
54
prodromal symptoms of erythema multiforme (EM)
1. fever
2. malaise
3. headache
4. cough
5. sore throat
55
previous studies showed that erythema multiforme (EM) has a male predilection but more recent studies show what?
a female predilection
56
how long do pts experience prodromal symptoms before onset of erythema multiforme (EM)?
~1 week
57
varying degrees of severity of erythema multiforme (EM)
1. EM minor
| 2. EM major
58
EM minor of erythema multiforme (EM) MUCOSA features
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
which locations are affected by EM minor of erythema multiforme (EM)?
1. skin (extremities)
| 2. mucosa (oral, conjunctival, genitourinary, respiratory)
60
what mucosa in the oral cavity is usually spared from EM minor of erythema multiforme (EM)?
1. gingiva
| 2. hard palate
61
EM minor of erythema multiforme (EM) SKIN features
1. variety of appearances "multiforme"
2. round, dusky-red patches on skin of extremities "target lesions"
3. bullae with necrotic centers
62
EM major of erythema multiforme (EM) clinical features
1. 2 or more mucosal sites in conjunction with skin lesions
| 2. ocular involvement can produce symblepheron
63
which sites are affected by EM major of erythema multiforme (EM) AND EM minor?
1. mucosal
2. lip
3. skin
64
different processes of erythema multiforme (EM) than EM major and minor
1. Stevens-Johnson syndrome (SJS)
| 2. toxic epidermal necrolysis (TEN)
65
Stevens-Johnson syndrome (SJS)
at least two mucosal sites plus skin involvement
66
toxic epidermal necrolysis (TEN) is also known as what?
Lyell's disease
67
toxic epidermal necrolysis (TEN)
diffuse bullous involvement of skin and mucosa
68
what is the difference between SJS and TEN?
degree of skin involvement and age
69
SJS has what percent of skin involvement and usually affects who compared to TEN?
<10% of skin invovlement and usually younger pt
70
TEN has what percent of skin involvement and usually affect who compared to SJS?
>30% skin involvement and usually over 60 y.o.
71
distinguishing features between EM and SJS/TEN
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
clinical features of SJS and TEN
1. within 2 weeks skin sloughing and flaccid bullae develop
| 2. pts may appear badly scalded - usually tx'd in burn unit
73
almost all SJS and TEN pts have what?
mucosal involvement esp oral
74
dx of EM, SJS and TEN are usually based on what?
clinical presentation
75
T/F: DIF and IIF are non-specific so it's not helpful except in ruling out other immune-mediated conditions
true
76
histo features of EM, SJS, TEN
1. subepithelial or intraepithelial vesicles
2. necrotic basal keratinocytes
3. mixed inflammatory cell infiltrate
4. may see perivascular inflammation
77
supportive therapy for erythema multiforme (EM)
1. discontinue causative drug!
2. systemic or topical steroids early on
3. IV re-hydration
4. topic anesthetic or analgesic for pain
78
tx of SJS and TEN
1. discontinue causative drug!
2. IV re-hydration
3. topical anesthetic or analgesic for pain
79
what has been implicated in inducing TEN?
NSAIDS
80
why should steroids be avoided in managing TEN?
associated with increased mortality
81
how does IV administration of pooled human immunoglobulins seems helpful in resolution of TEN?
by blocking apoptosis of epithelial cells
82
EM minor and major are self-limiting and should resolve in how many weeks?
2-6 wks
83
20% of EM minor and major pts get recurrences especially during what time(s)?
spring and autumn
84
if HSV triggered EM minor or major, what is indicated for maintenance?
antiviral
85
T/F: erythema multiforme (EM) is not life-threatening except when?
severe cases
86
mortality rate of SJS
1-5%
87
mortality rate of TEN
25-30%
88
px of pts that survives from SJS or TEN
1. skin heals in 3-5 wks
2. oral lesions take longer
3. ocular damage in 50% of pts
89
erythema migrans is also known as what?
1. geographic tongue
| 2. benign migratory glossitis
90
etiopathogenesis of erythema migrans is unknown but is probably what?
immune-mediated
91
erythema migrans is one ofhte more common oral conditions occurring in what percent of the population?
1-3% so considered "common oral lesion"
92
erythema seen in erythema migrans is due to what?
atrophy of filiform papillae and shearing off of the parakeratin causing epithelium to thin resulting in red appearance
93
erythema migrans occurs in 1/3rd of pts who have what?
fissured tongue
94
clinical features of erythema migrans
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
where does erythema migrans characteristically affect on the tongue?
dorsal and lateral anterior 2/3 of tongue
96
ectopic geographic tongue
erythema migrans on other non-keratinized mucosal surfaces
97
histo of erythema migrans is similar to what?
psoriasis "psoriasiform mucositis" description on bx report
98
histo of erythema migrans
1. parakeratosis with extensive microabscess (neutrophils) formation in superficial spinous layer
2. elongation of rete ridges
3. neutrophilic infiltration
99
tx of erythema migrans
none
100
occassionaly pts with erythema migrans are sensitive or painful to what?
hot, spicy foods... can use potent topical steroid
101
px of erythema migrans
good
102
T/F: pts with erythema migrans should be reassured that this is a benign condition
true
