Developmental and Immune-mediated Mucocutaneous Conditions - part III Flashcards
(88 cards)
1
Q
lichen planus (LP) is what type of disorder?
A
chronic immune-mediated
2
Q
CUTANEOUS lichen planus (LP) lesions may resolve in how many years?
A
7-10 yrs
3
Q
how is mucosal lichen planus (LP) lesions usually managed?
A
as chronic condition
4
Q
who is affected by CUTANEOUS lichen planus (LP)?
A
adults ~30-60 y.o.
5
Q
what gender predilection does CUTANEOUS lichen planus (LP) have?
A
female predilection
6
Q
clinical features of CUTANEOUS lichen planus (LP)
A
purple polygonal pruritic papules with Wickham’s striae (lacy white lines)
7
Q
CUTANEOUS lichen planus (LP) may affect any locations but which sites does it most commonly affect?
A
- flexor surface of wrists
- lumbar region
- shins
8
Q
what are the two forms of oral lichen planus (OLP)?
A
- reticular
2. erosive (ELP)
9
Q
reticular oral lichen planus (OLP)
A
lacy white lines
10
Q
erosive lichen planus (ELP)
A
erythematous, may ulcerate
11
Q
clinical features of oral lichen planus (OLP/ELP)
A
may occur alone or with skin lesions
12
Q
who does oral lichen planus (OLP) affect?
A
adults
13
Q
what is the gender predilection for oral lichen planus (OLP)?
A
3:2 female predilection
14
Q
what is the most common form of oral lichen planus (OLP)?
A
reticular
15
Q
what is the most symptomatic form of oral lichen planus (OLP)?
A
erosive especially with acidic, salty, or spicy foods
16
Q
clinical feature of reticular lichen planus
A
interlacing white lines
17
Q
clinical feature of erosive lichen planus
A
- shallow ulcers
2. peripheral erythema and radiating white lines
18
Q
sites most commonly affected by oral lichen planus (OLP)
A
- bilateral buccal mucosa
- tongue
- gingiva
- any intraoral surface
- lips
19
Q
how might oral lichen planus (OLP) appear if the dorsal tongue is involved?
A
patchy keratosis and atrophy
20
Q
what can be superimposed on either types of oral lichen planus (OLP)?
A
candida albicans
21
Q
what will worsen if oral lichen planus (OLP) is superimposed with candidal infection?
A
oral ulcerative conditions worsen
22
Q
T/F: histo features of oral lichen planus (OLP) are characteristic, but not specific
A
true
23
Q
histo features of oral lichen planus (OLP)
A
- varying degrees of hyperkeratosis, atrophy or ulceration
- absent or pointed rete ridges (“saw-toothed”)
- degeneration of basal cell layer (degenerating keratinocytes)
- band-line infiltrate of lymphocytes
- varying thickness of spinous layer
24
Q
DIF of oral lichen planus (OLP) is non-specific except to what?
A
rule out other immune-meidated condition
25
what can have the same histology as oral lichen planus (OLP)?
inflammatory response to epithelial dysplasia (especially mild) can have same histology as lichen planus
26
oral lichen planus (OLP) is a CLINICAL dx and should be correlated with what?
microscopic findings
27
what should be ruled out before tx'ing oral lichen planus (OLP)?
candidiasis; tx if + culture
28
tx for reticular lichen planus
no tx needed
29
how might pts with reticular lichen planus feel?
feel "rough" areas of hyperkeratosis, but no pain
30
tx of oral lichen planus (OLP)
1. ELP tx with potent topical steroid
| 2. bx any suspected pre-malignant/malignant lesion
31
T/F: systemic steroids are needed in tx'ing ELP
false, NOT needed
32
why does the px of oral lichen planus (OLP) vary?
1. some pts are well-controlled, other can be difficult to control
2. recurring candidiasis a challenge
33
what causes candidiasis to recur in pts with oral lichen planus (OLP)?
1. dry mouth
2. dentures
3. abx
4. inhaler
5. topical steroid predisposes to candida
34
why is there controversy surrounding malignant transformation of oral lichen planus (OLP)?
1. no molecular evidence supporting it
| 2. many cases of purported transformation poorly documented
35
lichenoid mucositis
number of conditions that can mimic oral lichen planus, both clinically and histopathologically
36
what does lichenoid mucositis entail?
1. lichenoid drug rxn
2. lichenoid amalgam rxn
3. oral mucosal cinnamon rxn
4. lichenoid foreign body gingivitis
5. oral lesions of graft vs host disease (GVHD)
6. oral lesions of lupus erythematosus (LE)
7. some epithelial dysplasia
37
what is the most common collagen vascular/CT diseases in US?
lupus erythematosus (LE)
38
what are the 3 forms of lupus erythematosus (LE)?
1. chronic cutaneous lupus erythematosus (CCLE)
2. systemic lupus erythematosus (SLE)
3. subacute cutaneous lupus erythematosis (SCLE) -intermediate b/w CCLE and SLE
39
where does chronic cutaneous lupus erythematosus (CCLE) primarily affect?
skin and mucosa
40
what is chronic cutaneous lupus erythematosus (CCLE) also known as?
"discoid lupus"
41
chronic cutaneous lupus erythematosus (CCLE) is exacerbated by what?
sun exposure
42
clinical features of chronic cutaneous lupus erythematosus (CCLE)
1. may have no signs or symptoms
| 2. waxing/waning nature
43
SKIN features of chronic cutaneous lupus erythematosus (CCLE)
scaly, erythematous patches in sun-exposed areas, esp. H&N
44
what might skin lesions from chronic cutaneous lupus erythematosus (CCLE) heal and then reappear in different area result in?
atrophy and scarring with hypo- or hyperpigmentation
45
chronic cutaneous lupus erythematosus (CCLE) lesions on mucosa is called what?
lichenoid mucositis
46
mucosal lesions on pt's with chronic cutaneous lupus erythematosus (CCLE) are identical to what?
ELP
47
T/F: mucosal lesions on pts with chronic cutaneous lupus erythematosus (CCLE) seldomly occur without skin lesions
true
48
mucosal lesions on pts with chronic cutaneous lupus erythematosus (CCLE) are painful especially when they eat what?
acidic, salty, or spicy foods
49
clinical features of systemic lupus erythematosus
1. waxing/waning nature
| 2. malar "butterfly rash" spares nasolabial folds
50
females are how many times more likely to be affected by systemic lupus erythematosus than males?
8-10x
51
women of what race are more likely to be affected by systemic lupus erythematosus?
women of color more than Caucasians
52
what is the average age of dx for systemic lupus erythematosus?
31 y.o.
53
what are the initial manifestations of systemic lupus erythematosus?
protean manifestations
54
protean manifestations for systemic lupus erythematosus
1. fever
2. weight loss
3. arthritis
4. fatigue
5. general malaise
55
when do skin lesions flare up on on pts with systemic lupus erythematosus?
with UV exposure
56
what is the most significant aspect of systemic lupus erythematosus?
renal involvement
57
what percent of pts with systemic lupus erythematosus have renal involvement?
40-50% of pts
58
what is common in pts with systemic lupus erythematosus?
cardiac involvement
59
cardiac involvement in pts with systemic lupus erythematosus
1. pericarditis
| 2. Libman-Sacks endocarditis
60
what percent of pts with systemic lupus erythematosus have oral involvement?
5-25%
61
ORAL features of systemic lupus erythematosus
may appear non-specific or lichenoid
62
where does systemic lupus erythematosus affect intraorally?
1. palate
2. buccal mucosa
3. gingiva
4. vermilion zones
63
systemic lupus erythematosus on vermilion zones
lupus cheilitis
64
histo featurs of CCLE and SLE
1. lichenoid features with vasculitis
| 2. may show subtle differences which may help lead to dx
65
what subtle differences can be seen histologically between CCLE and SLE?
subepithelial edema
66
T/F: dx of lupus erythematosus can be difficult especially early stages
true
67
how is lupus erythematosus dx'ed?
1. clinical appearance skin lesions in CCLE characteristic
2. serum studies show anti-nuclear antibodies (ANAs)
3. positive lupus band test
68
how is systemic lupus erythematosus (SLE) dx'ed?
criteria by American Rheumatism Association for clinical and lab findings
69
anti-nuclear antibodies (ANAs) measure what?
the amount and pattern of antibodies in your blood that work against your own body
70
a non-specific finding of ANAs can be used for what?
as a screening tool
71
ANAs are present in what percent of SLE pts?
95%
72
ANAs are present in what percent of CCLE pts?
negative
73
lupus band test
deposition of a band of immunoreactants at the basement membrane zone of normal skin
74
T/F: a positive lupus band test is specific to SLE
false, not specific...
is positive in other immune-mediated conditions
75
T/F: some patients with systemic lupus erythematosus are negative for the lupus band test
true
76
tx for lupus erythematosus
1. sunscreens, avoid excessive UV exposure
2. NSAIDS
3. antimalarial drug therapy
4. topical steroidsfor skin and/or mucosa
5. systemic steroids or immunomodulating agents for more severe cases
6. kidney transplant
77
antimalarial drug therapy that should be given to pts with lupus erythematosus if resistant to topical tx
low dose thalidomide
78
T/F: pts with systemic lupus erythematosus tx'd with systemic steroids or immunomodulating agents can have complications from long-term steroids
true
79
px of chronic cutaneous lupus erythematosus
good
80
~50% of pts with chronic cutaneous lupus erythematosus does what after several years?
resolve
81
what percent of pts with CCLE transform to SLE?
~5-10%
82
px of systemic lupus erythematosus
variable
83
px of pts with systemic lupus erythematosus depends on what?
1. which organs affected
| 2. how frequently SLE is reactivated
84
px for systemic lupus erythematosus is worse for men or women?
worse for men than women
85
px for systemic lupus erythematosus is worse for which race?
worse for blacks than whites
86
what is the most common cause of death in pts with systemic lupus erythematosus?
renal failure
87
what is the 5-year survival rate for systemic lupus erythematosus?
95%
88
what is the 15-year survival rate for systemic lupus erythematosus?
75%
