immune mediated mucocutaneous

Question 1

Pemphigus Vulgaris autoantibodes destory what

Answer 1

desmosomes

Question 2

Pemphigus Vulgaris happens in who

Answer 2

either sex
4th to 6th decade.
fatal if not treated

Question 3

Pemphigus Vulgaris clinical signs

Answer 3

>50 % have oral lesions
Ragged erosions and ulcerations
any mucosa surface
flaccid bullae on skin
Nikolsky sign

Question 4

oral lesion characteristics

Answer 4

“first to show, last to go”

Oral lesions are often the initial manifestation of the disease and the hardest to resolve with therapy

Question 5

normal tissue adjacent to ulcerated tissue should be tested with what kind of test

Answer 5

Direct immunofluorescense.

Question 6

both DIF and IFF will be positive or negative?

Answer 6

positive

Question 7

autoantibodies bind _______

Answer 7

desmosomes components (desmoglein 3 and 1)

Question 8

3 histopath features

Answer 8

1. Intraepithelial (aka suprabasilar) clefting
2. Acantholysis (breakdown of spinous
   layer) – is also usually evident
3. “Dilapidated brick wall” with intact basal cell layer

Question 9

treatment for pemphigus vulgaris

Answer 9

systemic corticosteroids.

Question 10

prior to corticosteroids the mortality rate was what

Answer 10

60-90%

Question 11

mortality rare today of PV

Answer 11

5-10% usually due to complication of therapy

Question 12

Pemphigoid, also called what

Answer 12

cicatricial pemphigoid

Question 13

Pemphigoid blisters resemble what other disease

Answer 13

Pemphigus

Question 14

Pemphigoid more or less common than pemphigus

Answer 14

2-4 times more common

Question 15

pemphigoid affects who more often

Answer 15

female 2:1, older age group 50-60s

Question 16

pemphigoid clinical

Answer 16

can affect any mucosa surface, sometimes skin
scarring usually seen (skin and ocular)
desquamative gingivitis
may see intact blisters intraorally

Question 17

most significant aspect of the disease is _____ involvement

Answer 17

ocular

Question 18

what does ocular scaring do to the eye, how does it cause blindness

Answer 18

• 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

Question 19

histopath features

Answer 19

subepithelium cleft formation

separation of the intact epithelium from the connective tissue at the BMZ

Question 20

submit normal tissue how far away from ulcerations

Answer 20

.5 -1 cm away

Question 21

tissue should be submitted in what kind of solutions

Answer 21

michaels solution and formalin

Question 22

DIF is usually ______ IIF is usually _______

Answer 22

positive, negative

Question 23

see linear depositions of immunorecatants at the _____

Answer 23

BMZ

Question 24

pemphigoid treatment

Answer 24

depends on severity of disease
oral lesions only you can use topical steroids
if ocular involvement systemic immunosuppressive therapy is indicated.

Question 25

pemphigoid prognosis

Answer 25

rarely fatal condition can usually be controlled blindness can occur if you don't treat ocular scaring. rarely self resolve

Question 26

Bullous Pemphigoid in who most often

Answer 26

older adults 75-80

Question 27

bullous pemphigoid clinical

Answer 27

cutaneous lesions mostly 10-20% oral involvement Pruritus is common initial complaint, followed by cutaneous blisters

Question 28

bullous pemphigoid similarities to MM pemphigoid

Answer 28

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

Question 29

bullous pemphigoid differences to MM pemphigoid

Answer 29

Positive IIF | can self resolve in 1-2 years

Question 30

Erythema Multiforme

Answer 30

Acute, self-limiting ulcerative disorder Probably immune-mediated

Question 31

etiology of erythema multiforme

Answer 31

50% unknown 50% most are infection-related (often HSV) or (less commonly) medication-related

Question 32

who is this found in the most

Answer 32

young adult females

Question 33

EM has a spectrum of disease, EM minor

Answer 33

skin or oral mucosa only

Question 34

EM major

Answer 34

at least two mucosal sites plus skin involvement

Question 35

Stevens-Johnson syndrome & toxic epidermal necrolysis

Answer 35

diffuse involvement of skin and mucosa | Stevens-Johnson 30%

Question 36

EM – Clinical Features

Answer 36

1. Hemorrhagic crusting of lips 2. Widespread oral ulcers with ragged margins 3. Labial mucosa, buccal mucosa and tongue 4. “Target” lesions of skin

Question 37

Histopathology of EM

Answer 37

not diagnostic – light microscopic features | Keratinocyte destruction; subepithelial edema; mixed inflammatory infiltrate; perivascular inflammation

Question 38

EM treatment

Answer 38

mild- supportive care major- corticosteroids TEN managed in burn unit; IV pooled human immunoglobulin shows promise

Question 39

EM - Prognosis

Answer 39

mild to moderate- good major- 1-5% mortality TEN- 25-30% historically; IV Ig therapy has dramatically improved patient recovery

Question 40

EM recurrence

Answer 40

may be recurrent in 20% of cases

Question 41

Erythema migrans also called what

Answer 41

benign migratory glossitis or geographic tongue occuring in 1-3% of the population immune related.

Question 42

erythema migrants is often seen with what

Answer 42

fissured tongue | waxes and wanes

Question 43

May develop on other non-keratinized mucosal surfaces –called what?

Answer 43

“ectopic geographic tongue”

Question 44

histopath of erythema migrants, similar to what

Answer 44

psoriasis Parakeratosis with extensive microabscess formation in the upper spinous layer

Question 45

Parakeratosis with extensive microabscess formation in the upper spinous layer results in what

Answer 45

loss of superficial parakeratin | Remaining epithelium is much thinner and appears red

Question 46

treatment for erythema migrants

Answer 46

no treatment is generally necessary | some patients complain of sensitivity to hot or spicy foods when lesions are active.