immune mediated mucocutaneous Flashcards
Pemphigus Vulgaris autoantibodes destory what
desmosomes
Pemphigus Vulgaris happens in who
either sex
4th to 6th decade.
fatal if not treated
Pemphigus Vulgaris clinical signs
>50 % have oral lesions Ragged erosions and ulcerations any mucosa surface flaccid bullae on skin Nikolsky sign
oral lesion characteristics
“first to show, last to go”
Oral lesions are often the initial manifestation of the disease and the hardest to resolve with therapy
normal tissue adjacent to ulcerated tissue should be tested with what kind of test
Direct immunofluorescense.
both DIF and IFF will be positive or negative?
positive
autoantibodies bind _______
desmosomes components (desmoglein 3 and 1)
3 histopath features
- Intraepithelial (aka suprabasilar) clefting
- Acantholysis (breakdown of spinous
layer) – is also usually evident - “Dilapidated brick wall” with intact basal cell layer
treatment for pemphigus vulgaris
systemic corticosteroids.
prior to corticosteroids the mortality rate was what
60-90%
mortality rare today of PV
5-10% usually due to complication of therapy
Pemphigoid, also called what
cicatricial pemphigoid
Pemphigoid blisters resemble what other disease
Pemphigus
Pemphigoid more or less common than pemphigus
2-4 times more common
pemphigoid affects who more often
female 2:1, older age group 50-60s
pemphigoid clinical
can affect any mucosa surface, sometimes skin
scarring usually seen (skin and ocular)
desquamative gingivitis
may see intact blisters intraorally
most significant aspect of the disease is _____ involvement
ocular
what does ocular scaring do to the eye, how does it cause blindness
- Scarring obstructs the orifices of glands that produce the tear film, resulting in a dry eye
- Dryness leads to keratinization of the corneal epithelium, leading to blindness
- Scarring may lead to adhesion formation (symblepharons) between eyelid and globe
histopath features
subepithelium cleft formation
separation of the intact epithelium from the connective tissue at the BMZ
submit normal tissue how far away from ulcerations
.5 -1 cm away
tissue should be submitted in what kind of solutions
michaels solution and formalin
DIF is usually ______ IIF is usually _______
positive, negative
see linear depositions of immunorecatants at the _____
BMZ
pemphigoid treatment
depends on severity of disease
oral lesions only you can use topical steroids
if ocular involvement systemic immunosuppressive therapy is indicated.
pemphigoid prognosis
rarely fatal
condition can usually be controlled
blindness can occur if you don’t treat ocular scaring.
rarely self resolve
Bullous Pemphigoid in who most often
older adults 75-80
bullous pemphigoid clinical
cutaneous lesions mostly
10-20% oral involvement
Pruritus is common initial complaint, followed by cutaneous blisters
bullous pemphigoid similarities to MM pemphigoid
Subepithelial cleft similar to MM pemphigoid
Positive DIF and IIF, immunoreactants deposited at the BMZ
Management similar to MM pemphigoid but immunosuppressive therapy can have serious side effects
bullous pemphigoid differences to MM pemphigoid
Positive IIF
can self resolve in 1-2 years
Erythema Multiforme
Acute, self-limiting ulcerative disorder Probably immune-mediated
etiology of erythema multiforme
50% unknown
50% most are infection-related (often HSV) or
(less commonly) medication-related
who is this found in the most
young adult females
EM has a spectrum of disease, EM minor
skin or oral mucosa only
EM major
at least two mucosal sites plus skin involvement
Stevens-Johnson syndrome & toxic epidermal necrolysis
diffuse involvement of skin and mucosa
Stevens-Johnson 30%
EM – Clinical Features
- Hemorrhagic crusting of lips
- Widespread oral ulcers with ragged margins
- Labial mucosa, buccal mucosa and tongue
- “Target” lesions of skin
Histopathology of EM
not diagnostic – light microscopic features
Keratinocyte destruction; subepithelial edema; mixed inflammatory infiltrate; perivascular inflammation
EM treatment
mild- supportive care
major- corticosteroids
TEN managed in burn unit; IV pooled human immunoglobulin shows promise
EM - Prognosis
mild to moderate- good
major- 1-5% mortality
TEN- 25-30% historically; IV Ig therapy has dramatically improved patient recovery
EM recurrence
may be recurrent in 20% of cases
Erythema migrans also called what
benign migratory glossitis or geographic tongue
occuring in 1-3% of the population
immune related.
erythema migrants is often seen with what
fissured tongue
waxes and wanes
May develop on other non-keratinized mucosal surfaces –called what?
“ectopic geographic tongue”
histopath of erythema migrants, similar to what
psoriasis
Parakeratosis with extensive microabscess formation in the upper spinous layer
Parakeratosis with extensive microabscess formation in the upper spinous layer results in what
loss of superficial parakeratin
Remaining epithelium is much thinner and appears red
treatment for erythema migrants
no treatment is generally necessary
some patients complain of sensitivity to hot or spicy foods when lesions are active.
