Developmental and Immune-mediated Mucocutaneous Conditions - part III Flashcards
lichen planus (LP) is what type of disorder?
chronic immune-mediated
CUTANEOUS lichen planus (LP) lesions may resolve in how many years?
7-10 yrs
how is mucosal lichen planus (LP) lesions usually managed?
as chronic condition
who is affected by CUTANEOUS lichen planus (LP)?
adults ~30-60 y.o.
what gender predilection does CUTANEOUS lichen planus (LP) have?
female predilection
clinical features of CUTANEOUS lichen planus (LP)
purple polygonal pruritic papules with Wickham’s striae (lacy white lines)
CUTANEOUS lichen planus (LP) may affect any locations but which sites does it most commonly affect?
- flexor surface of wrists
- lumbar region
- shins
what are the two forms of oral lichen planus (OLP)?
- reticular
2. erosive (ELP)
reticular oral lichen planus (OLP)
lacy white lines
erosive lichen planus (ELP)
erythematous, may ulcerate
clinical features of oral lichen planus (OLP/ELP)
may occur alone or with skin lesions
who does oral lichen planus (OLP) affect?
adults
what is the gender predilection for oral lichen planus (OLP)?
3:2 female predilection
what is the most common form of oral lichen planus (OLP)?
reticular
what is the most symptomatic form of oral lichen planus (OLP)?
erosive especially with acidic, salty, or spicy foods
clinical feature of reticular lichen planus
interlacing white lines
clinical feature of erosive lichen planus
- shallow ulcers
2. peripheral erythema and radiating white lines
sites most commonly affected by oral lichen planus (OLP)
- bilateral buccal mucosa
- tongue
- gingiva
- any intraoral surface
- lips
how might oral lichen planus (OLP) appear if the dorsal tongue is involved?
patchy keratosis and atrophy
what can be superimposed on either types of oral lichen planus (OLP)?
candida albicans
what will worsen if oral lichen planus (OLP) is superimposed with candidal infection?
oral ulcerative conditions worsen
T/F: histo features of oral lichen planus (OLP) are characteristic, but not specific
true
histo features of oral lichen planus (OLP)
- 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
DIF of oral lichen planus (OLP) is non-specific except to what?
rule out other immune-meidated condition
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
oral lichen planus (OLP) is a CLINICAL dx and should be correlated with what?
microscopic findings
what should be ruled out before tx’ing oral lichen planus (OLP)?
candidiasis; tx if + culture
tx for reticular lichen planus
no tx needed
how might pts with reticular lichen planus feel?
feel “rough” areas of hyperkeratosis, but no pain
tx of oral lichen planus (OLP)
- ELP tx with potent topical steroid
2. bx any suspected pre-malignant/malignant lesion
T/F: systemic steroids are needed in tx’ing ELP
false, NOT needed
why does the px of oral lichen planus (OLP) vary?
- some pts are well-controlled, other can be difficult to control
- recurring candidiasis a challenge
what causes candidiasis to recur in pts with oral lichen planus (OLP)?
- dry mouth
- dentures
- abx
- inhaler
- topical steroid predisposes to candida
why is there controversy surrounding malignant transformation of oral lichen planus (OLP)?
- no molecular evidence supporting it
2. many cases of purported transformation poorly documented
lichenoid mucositis
number of conditions that can mimic oral lichen planus, both clinically and histopathologically
what does lichenoid mucositis entail?
- lichenoid drug rxn
- lichenoid amalgam rxn
- oral mucosal cinnamon rxn
- lichenoid foreign body gingivitis
- oral lesions of graft vs host disease (GVHD)
- oral lesions of lupus erythematosus (LE)
- some epithelial dysplasia
what is the most common collagen vascular/CT diseases in US?
lupus erythematosus (LE)
what are the 3 forms of lupus erythematosus (LE)?
- chronic cutaneous lupus erythematosus (CCLE)
- systemic lupus erythematosus (SLE)
- subacute cutaneous lupus erythematosis (SCLE) -intermediate b/w CCLE and SLE
where does chronic cutaneous lupus erythematosus (CCLE) primarily affect?
skin and mucosa
what is chronic cutaneous lupus erythematosus (CCLE) also known as?
“discoid lupus”
chronic cutaneous lupus erythematosus (CCLE) is exacerbated by what?
sun exposure
clinical features of chronic cutaneous lupus erythematosus (CCLE)
- may have no signs or symptoms
2. waxing/waning nature
SKIN features of chronic cutaneous lupus erythematosus (CCLE)
scaly, erythematous patches in sun-exposed areas, esp. H&N
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
chronic cutaneous lupus erythematosus (CCLE) lesions on mucosa is called what?
lichenoid mucositis
mucosal lesions on pt’s with chronic cutaneous lupus erythematosus (CCLE) are identical to what?
ELP
T/F: mucosal lesions on pts with chronic cutaneous lupus erythematosus (CCLE) seldomly occur without skin lesions
true
mucosal lesions on pts with chronic cutaneous lupus erythematosus (CCLE) are painful especially when they eat what?
acidic, salty, or spicy foods
clinical features of systemic lupus erythematosus
- waxing/waning nature
2. malar “butterfly rash” spares nasolabial folds
females are how many times more likely to be affected by systemic lupus erythematosus than males?
8-10x
women of what race are more likely to be affected by systemic lupus erythematosus?
women of color more than Caucasians
what is the average age of dx for systemic lupus erythematosus?
31 y.o.
what are the initial manifestations of systemic lupus erythematosus?
protean manifestations
protean manifestations for systemic lupus erythematosus
- fever
- weight loss
- arthritis
- fatigue
- general malaise
when do skin lesions flare up on on pts with systemic lupus erythematosus?
with UV exposure
what is the most significant aspect of systemic lupus erythematosus?
renal involvement
what percent of pts with systemic lupus erythematosus have renal involvement?
40-50% of pts
what is common in pts with systemic lupus erythematosus?
cardiac involvement
cardiac involvement in pts with systemic lupus erythematosus
- pericarditis
2. Libman-Sacks endocarditis
what percent of pts with systemic lupus erythematosus have oral involvement?
5-25%
ORAL features of systemic lupus erythematosus
may appear non-specific or lichenoid
where does systemic lupus erythematosus affect intraorally?
- palate
- buccal mucosa
- gingiva
- vermilion zones
systemic lupus erythematosus on vermilion zones
lupus cheilitis
histo featurs of CCLE and SLE
- lichenoid features with vasculitis
2. may show subtle differences which may help lead to dx
what subtle differences can be seen histologically between CCLE and SLE?
subepithelial edema
T/F: dx of lupus erythematosus can be difficult especially early stages
true
how is lupus erythematosus dx’ed?
- clinical appearance skin lesions in CCLE characteristic
- serum studies show anti-nuclear antibodies (ANAs)
- positive lupus band test
how is systemic lupus erythematosus (SLE) dx’ed?
criteria by American Rheumatism Association for clinical and lab findings
anti-nuclear antibodies (ANAs) measure what?
the amount and pattern of antibodies in your blood that work against your own body
a non-specific finding of ANAs can be used for what?
as a screening tool
ANAs are present in what percent of SLE pts?
95%
ANAs are present in what percent of CCLE pts?
negative
lupus band test
deposition of a band of immunoreactants at the basement membrane zone of normal skin
T/F: a positive lupus band test is specific to SLE
false, not specific…
is positive in other immune-mediated conditions
T/F: some patients with systemic lupus erythematosus are negative for the lupus band test
true
tx for lupus erythematosus
- sunscreens, avoid excessive UV exposure
- NSAIDS
- antimalarial drug therapy
- topical steroidsfor skin and/or mucosa
- systemic steroids or immunomodulating agents for more severe cases
- kidney transplant
antimalarial drug therapy that should be given to pts with lupus erythematosus if resistant to topical tx
low dose thalidomide
T/F: pts with systemic lupus erythematosus tx’d with systemic steroids or immunomodulating agents can have complications from long-term steroids
true
px of chronic cutaneous lupus erythematosus
good
~50% of pts with chronic cutaneous lupus erythematosus does what after several years?
resolve
what percent of pts with CCLE transform to SLE?
~5-10%
px of systemic lupus erythematosus
variable
px of pts with systemic lupus erythematosus depends on what?
- which organs affected
2. how frequently SLE is reactivated
px for systemic lupus erythematosus is worse for men or women?
worse for men than women
px for systemic lupus erythematosus is worse for which race?
worse for blacks than whites
what is the most common cause of death in pts with systemic lupus erythematosus?
renal failure
what is the 5-year survival rate for systemic lupus erythematosus?
95%
what is the 15-year survival rate for systemic lupus erythematosus?
75%