Developmental and Immune-mediated Mucocutaneous Conditions - part III Flashcards

1
Q

lichen planus (LP) is what type of disorder?

A

chronic immune-mediated

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2
Q

CUTANEOUS lichen planus (LP) lesions may resolve in how many years?

A

7-10 yrs

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3
Q

how is mucosal lichen planus (LP) lesions usually managed?

A

as chronic condition

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4
Q

who is affected by CUTANEOUS lichen planus (LP)?

A

adults ~30-60 y.o.

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5
Q

what gender predilection does CUTANEOUS lichen planus (LP) have?

A

female predilection

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6
Q

clinical features of CUTANEOUS lichen planus (LP)

A

purple polygonal pruritic papules with Wickham’s striae (lacy white lines)

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7
Q

CUTANEOUS lichen planus (LP) may affect any locations but which sites does it most commonly affect?

A
  1. flexor surface of wrists
  2. lumbar region
  3. shins
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8
Q

what are the two forms of oral lichen planus (OLP)?

A
  1. reticular

2. erosive (ELP)

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9
Q

reticular oral lichen planus (OLP)

A

lacy white lines

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10
Q

erosive lichen planus (ELP)

A

erythematous, may ulcerate

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11
Q

clinical features of oral lichen planus (OLP/ELP)

A

may occur alone or with skin lesions

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12
Q

who does oral lichen planus (OLP) affect?

A

adults

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13
Q

what is the gender predilection for oral lichen planus (OLP)?

A

3:2 female predilection

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14
Q

what is the most common form of oral lichen planus (OLP)?

A

reticular

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15
Q

what is the most symptomatic form of oral lichen planus (OLP)?

A

erosive especially with acidic, salty, or spicy foods

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16
Q

clinical feature of reticular lichen planus

A

interlacing white lines

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17
Q

clinical feature of erosive lichen planus

A
  1. shallow ulcers

2. peripheral erythema and radiating white lines

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18
Q

sites most commonly affected by oral lichen planus (OLP)

A
  1. bilateral buccal mucosa
  2. tongue
  3. gingiva
  4. any intraoral surface
  5. lips
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19
Q

how might oral lichen planus (OLP) appear if the dorsal tongue is involved?

A

patchy keratosis and atrophy

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20
Q

what can be superimposed on either types of oral lichen planus (OLP)?

A

candida albicans

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21
Q

what will worsen if oral lichen planus (OLP) is superimposed with candidal infection?

A

oral ulcerative conditions worsen

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22
Q

T/F: histo features of oral lichen planus (OLP) are characteristic, but not specific

A

true

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23
Q

histo features of oral lichen planus (OLP)

A
  1. varying degrees of hyperkeratosis, atrophy or ulceration
  2. absent or pointed rete ridges (“saw-toothed”)
  3. degeneration of basal cell layer (degenerating keratinocytes)
  4. band-line infiltrate of lymphocytes
  5. varying thickness of spinous layer
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24
Q

DIF of oral lichen planus (OLP) is non-specific except to what?

A

rule out other immune-meidated condition

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25
Q

what can have the same histology as oral lichen planus (OLP)?

A

inflammatory response to epithelial dysplasia (especially mild) can have same histology as lichen planus

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26
Q

oral lichen planus (OLP) is a CLINICAL dx and should be correlated with what?

A

microscopic findings

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27
Q

what should be ruled out before tx’ing oral lichen planus (OLP)?

A

candidiasis; tx if + culture

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28
Q

tx for reticular lichen planus

A

no tx needed

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29
Q

how might pts with reticular lichen planus feel?

A

feel “rough” areas of hyperkeratosis, but no pain

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30
Q

tx of oral lichen planus (OLP)

A
  1. ELP tx with potent topical steroid

2. bx any suspected pre-malignant/malignant lesion

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31
Q

T/F: systemic steroids are needed in tx’ing ELP

A

false, NOT needed

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32
Q

why does the px of oral lichen planus (OLP) vary?

A
  1. some pts are well-controlled, other can be difficult to control
  2. recurring candidiasis a challenge
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33
Q

what causes candidiasis to recur in pts with oral lichen planus (OLP)?

A
  1. dry mouth
  2. dentures
  3. abx
  4. inhaler
  5. topical steroid predisposes to candida
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34
Q

why is there controversy surrounding malignant transformation of oral lichen planus (OLP)?

A
  1. no molecular evidence supporting it

2. many cases of purported transformation poorly documented

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35
Q

lichenoid mucositis

A

number of conditions that can mimic oral lichen planus, both clinically and histopathologically

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36
Q

what does lichenoid mucositis entail?

A
  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
Q

what is the most common collagen vascular/CT diseases in US?

A

lupus erythematosus (LE)

38
Q

what are the 3 forms of lupus erythematosus (LE)?

A
  1. chronic cutaneous lupus erythematosus (CCLE)
  2. systemic lupus erythematosus (SLE)
  3. subacute cutaneous lupus erythematosis (SCLE) -intermediate b/w CCLE and SLE
39
Q

where does chronic cutaneous lupus erythematosus (CCLE) primarily affect?

A

skin and mucosa

40
Q

what is chronic cutaneous lupus erythematosus (CCLE) also known as?

A

“discoid lupus”

41
Q

chronic cutaneous lupus erythematosus (CCLE) is exacerbated by what?

A

sun exposure

42
Q

clinical features of chronic cutaneous lupus erythematosus (CCLE)

A
  1. may have no signs or symptoms

2. waxing/waning nature

43
Q

SKIN features of chronic cutaneous lupus erythematosus (CCLE)

A

scaly, erythematous patches in sun-exposed areas, esp. H&N

44
Q

what might skin lesions from chronic cutaneous lupus erythematosus (CCLE) heal and then reappear in different area result in?

A

atrophy and scarring with hypo- or hyperpigmentation

45
Q

chronic cutaneous lupus erythematosus (CCLE) lesions on mucosa is called what?

A

lichenoid mucositis

46
Q

mucosal lesions on pt’s with chronic cutaneous lupus erythematosus (CCLE) are identical to what?

A

ELP

47
Q

T/F: mucosal lesions on pts with chronic cutaneous lupus erythematosus (CCLE) seldomly occur without skin lesions

A

true

48
Q

mucosal lesions on pts with chronic cutaneous lupus erythematosus (CCLE) are painful especially when they eat what?

A

acidic, salty, or spicy foods

49
Q

clinical features of systemic lupus erythematosus

A
  1. waxing/waning nature

2. malar “butterfly rash” spares nasolabial folds

50
Q

females are how many times more likely to be affected by systemic lupus erythematosus than males?

A

8-10x

51
Q

women of what race are more likely to be affected by systemic lupus erythematosus?

A

women of color more than Caucasians

52
Q

what is the average age of dx for systemic lupus erythematosus?

A

31 y.o.

53
Q

what are the initial manifestations of systemic lupus erythematosus?

A

protean manifestations

54
Q

protean manifestations for systemic lupus erythematosus

A
  1. fever
  2. weight loss
  3. arthritis
  4. fatigue
  5. general malaise
55
Q

when do skin lesions flare up on on pts with systemic lupus erythematosus?

A

with UV exposure

56
Q

what is the most significant aspect of systemic lupus erythematosus?

A

renal involvement

57
Q

what percent of pts with systemic lupus erythematosus have renal involvement?

A

40-50% of pts

58
Q

what is common in pts with systemic lupus erythematosus?

A

cardiac involvement

59
Q

cardiac involvement in pts with systemic lupus erythematosus

A
  1. pericarditis

2. Libman-Sacks endocarditis

60
Q

what percent of pts with systemic lupus erythematosus have oral involvement?

A

5-25%

61
Q

ORAL features of systemic lupus erythematosus

A

may appear non-specific or lichenoid

62
Q

where does systemic lupus erythematosus affect intraorally?

A
  1. palate
  2. buccal mucosa
  3. gingiva
  4. vermilion zones
63
Q

systemic lupus erythematosus on vermilion zones

A

lupus cheilitis

64
Q

histo featurs of CCLE and SLE

A
  1. lichenoid features with vasculitis

2. may show subtle differences which may help lead to dx

65
Q

what subtle differences can be seen histologically between CCLE and SLE?

A

subepithelial edema

66
Q

T/F: dx of lupus erythematosus can be difficult especially early stages

A

true

67
Q

how is lupus erythematosus dx’ed?

A
  1. clinical appearance skin lesions in CCLE characteristic
  2. serum studies show anti-nuclear antibodies (ANAs)
  3. positive lupus band test
68
Q

how is systemic lupus erythematosus (SLE) dx’ed?

A

criteria by American Rheumatism Association for clinical and lab findings

69
Q

anti-nuclear antibodies (ANAs) measure what?

A

the amount and pattern of antibodies in your blood that work against your own body

70
Q

a non-specific finding of ANAs can be used for what?

A

as a screening tool

71
Q

ANAs are present in what percent of SLE pts?

A

95%

72
Q

ANAs are present in what percent of CCLE pts?

A

negative

73
Q

lupus band test

A

deposition of a band of immunoreactants at the basement membrane zone of normal skin

74
Q

T/F: a positive lupus band test is specific to SLE

A

false, not specific…

is positive in other immune-mediated conditions

75
Q

T/F: some patients with systemic lupus erythematosus are negative for the lupus band test

A

true

76
Q

tx for lupus erythematosus

A
  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
Q

antimalarial drug therapy that should be given to pts with lupus erythematosus if resistant to topical tx

A

low dose thalidomide

78
Q

T/F: pts with systemic lupus erythematosus tx’d with systemic steroids or immunomodulating agents can have complications from long-term steroids

A

true

79
Q

px of chronic cutaneous lupus erythematosus

A

good

80
Q

~50% of pts with chronic cutaneous lupus erythematosus does what after several years?

A

resolve

81
Q

what percent of pts with CCLE transform to SLE?

A

~5-10%

82
Q

px of systemic lupus erythematosus

A

variable

83
Q

px of pts with systemic lupus erythematosus depends on what?

A
  1. which organs affected

2. how frequently SLE is reactivated

84
Q

px for systemic lupus erythematosus is worse for men or women?

A

worse for men than women

85
Q

px for systemic lupus erythematosus is worse for which race?

A

worse for blacks than whites

86
Q

what is the most common cause of death in pts with systemic lupus erythematosus?

A

renal failure

87
Q

what is the 5-year survival rate for systemic lupus erythematosus?

A

95%

88
Q

what is the 15-year survival rate for systemic lupus erythematosus?

A

75%