Developmental and Immune-mediated Mucocutaneous Conditions - part I COPY Flashcards
categories of mucocutaneous conditions
- developmental
2. immune-mediated
developmental mucocutaneous conditions
- ectodermal dysplasia
- white sponge nevus
- Peutz-Jeghers syndrome
- Hereditary hemorrhagic telangiectasia
immune-mediated mucocutaneous conditions
- pemphigus vulgaris
- mucous membrane pemphigoid
- bullous pemphigoid
- erythema multiforme
- erythema migrans (geographic tongue)
- linchen planus
- lichenoid mucositis
- lupus erthematosus
- systemic sclerosis
- CREST syndrome
etiology of pemphigus vulgaris (PV)
autoimmune
Pemphigus vulgaris (PV) is due to inappropriate production of what?
antibodies by the host directed against host tissue (autoantibodies)…
damage to host by host’s own immune response
Pemphigus vulgaris (PV) autoantibodies destroy what?
desmosomes
role of desmosomes
bond epithelial cells together
what happens when antibodies inhibit desmosomes?
inhibits adherence so a split develops in the epithelium
T/F: Pemphigus vulgaris (PV) is relatively rare
true, ~5 cases per million dx’d each year in general population
what is the average age of pts dx’d with pemphigus vulgaris (PV)?
50 y.o.
what is the gender predilection for pemphigus vulgaris (PV)?
no gender predilection
oral lesions in pts with pemphigus vulgaris (PV)
- superficial, ragged erosions and ulcerations
2. any mucosal surface
T/F: the oral lesions in pts with pemphigus vulgaris (PV) are the “first to show, last to go”
true, oral lesions are the initial manifestation of the disease and the most difficult to resolve with treatment
clinical features of pemphigus vulgaris (PV)
- flaccid vesicles, bullae on skin
2. + Nikolsky sign
+ Nikolsky sign
inducing a bulla by applying firm, lateral pressure to normal appearing skin
what is rarely seen intact intraorally in pts with pemphigus vulgaris (PV)?
flaccid vesicles, bullae
what is important in managing pemphigus vulgaris (PV)?
plaque control
normal tissue adjacent to ulceration or erosion of pemphigus vulgaris (PV) should be sampled for what?
- direct immunoflorescent (in Michel’s solution)
2. light microscopic evaluation
histo of pemphigus vulgaris (PV)
- INTRAEPITHELIAL clefting above the basal layer (i.e. within the epithelium)
- acantholysis
acantholysis
breakdown of spinous layer; cells appear to fall apart
what remains attached to the CT seen microscopically in pemphigus vulgaris (PV)?
only basal cell layer of epithelium
immunopathology identifies what?
immune responses associated with diseases
immunofluorescence
technique that uses fluorescent-labeled antibodies to detect specific targets
T/F: immunopathology is used to help identify several immune-mediated conditions
true
direct immunofluorescence (DIF) is used to detect what?
autoantibodies bound to pt’s tissues
indirect immunofluorescence (IIF) is used to detect what?
antibodies circulating in the blood
immuno features of pemphigus vulgaris (PV)
- both DIF and IIF studies will be positive in pemphigus vulgaris
- autoantibodies bind desmosomal components (desmoglein 1 & 3)
DIF-immunoreactants are what in pemphigus vulgaris (PV)?
in intercellular areas b/w the surface epithelial cells “chicken wire” with SUPRAbasilar cleft
tx of pemphigus vulgaris (PV)
systemic corticosteroids - often azathiprine or other steroid-sparing agents
why does topical corticosteroids have little effect in tx’ing pemphigus vulgaris (PV)?
PV is a systemic disease
what percent of pemphigus vulgaris (PV) cases will resolve on their own after 10 years?
~30%
T/F: pemphigus vulgaris (PV) is usually fatal if not tx’d
true
why may pemphigus vulgaris (PV) be fatal if not tx’d?
- severe infection
- loss of fluids/electrolytes
- malnutrition due to mouth pain
prior to corticosteroid therapy, what mortality rates of pemphigus vulgaris (PV) cases were what percent?
60-90%
what may lead to mortality of pts with pemphigus vulgaris (PV)?
complications of long-term steroid
what is the morality rate of pts with pemphigus vulgaris (PV) today?
5-10% mortality
mortality today in pts with pemphigus vulgaris (PV) is usually due to what?
complications of therapy (side effect of steroids, immune-suppression)
what drug is used to tx pemphigus vulgaris (PV) suppresses bone marrow and is a carcinogen?
azathioprine
