Immune-mediated Mucocutaneous Disease 1

Question 1

Which of these mucocutaneous lesions is immune-mediated?

• ectodermal dysplasia
• white sponge nevus
• lichen planus
• Peutz-Jeghers

Answer 1

lichen planus

Question 2

True or False: With pemphigus vulgaris, the autoantibodies destroy hemidesmosomes.

Answer 2

False, AB destroy desmosomes

Question 3

True or False: Pemphigus vulgaris has no sex predilection.

Answer 3

True, PV…pemphigus vulgaris…PV…penis or vagina

Question 4

Pemphigus Vulgaris is a rare immune mediated condition that is _____ if not treated.

Answer 4

fatal

Question 5

Why is PV fatal when not treated?

Answer 5

SEVERE INFECTION:

• loss of fluids
• malnutrition due to mouth pain

Question 6

How often do PV patients present with oral lesions? What do oral lesions look like?

Answer 6

50%
superficial, ragged erosions and ulcerations
any mucosal surface
oral blisters seen rarely

Question 7

PV lesions on the skin appear as _______ _____.

Answer 7

flaccid bullae

nikolsky sign

Question 8

Describe the presentation of a positive Nikolsky sign.

Answer 8

apply firm, lateral pressure to normal appearing skin

= induces a bulla

Question 9

True or False: The skin bullae with PV are the “first to show and last to go”

Answer 9

False, the oral lesions are first

oral: initial manifestation and most difficult to resolve

Question 10

What is immunofluorescence and its sub-categories?

Answer 10

a technique that uses fluorescent-labeled antibodies to detect specific targets

• DIF (direct)
• IFF (indirect)

Question 11

What is Direct Immunofluorescence used for?

Answer 11

to detect autoantibodies bound to the patient’s tissues

fluoro-IgG + patient biopsy–> microscopy

Question 12

What is Indirect Immunofluorescence used for?

Answer 12

to detect antibodies circulating in the blood

patient serum + control tissue + fluoro-IgG–> microscopy

Question 13

True or False: Both direct and indirect immunofluorescence will be negative in pemphigus vulgaris.

Answer 13

False, both DIF and IFF are positive in PV

Question 14

Normal tissue adjacent to PV ulceration should be sampled for _____.

Answer 14

DIF

Question 15

Autoantibodies in PV will bind to ______ components.

Answer 15

desmosomal (desmoglein 1 & desmoglein 3)

Question 16

Where should the PV sample be obtained for biopsy?

Answer 16

Lesions of PV should be sampled at the periphery, no the ulcerated center

Question 17

What is the microscopic feature that is always present with PV? What is the feature that is usually present?

Answer 17

always: intraepithelial clefting above the basal layer (within the epithelium)
often: acantholysis (breakdown of spinous layer, cells appear to fall apart)

Question 18

How is Pemphigus Vulgaris treated?

Answer 18

• systemic corticosteroids: often azathioprine or other steroid-sparing agents
• topical corticosteroids have little effect*

Question 19

True or False: PV is treated with either systemic or topical corticosteroids, whichever they prefer to use.

Answer 19

False, only systemic will work

Question 20

What is the mortality rate of PV? What was the rate prior to corticosteroid therapy?

Answer 20

5-10% mortality (complications of therapy)

60-90% mortality (prior to meds)

Question 21

Which immune-mediated mucocutaneous lesion has a similar clinical presentation to PV but is more than 2 times as common?

Answer 21

Mucous Membrane Pemphigoid

Question 22

Mucous Membrane Pemphigoid is also known as _______ pemphigoid, which means “scarring.”

Answer 22

cicatricial

Question 23

MMP has a ______ predilection with an average age of _____ years.

Answer 23

female (2:1)

50-60

Question 24

True or False: MMP may affect any mucosal surface and occasionally the skin.

Answer 24

True

Question 25

Why is MMP also refered to as Cicatricial Pemphigoid?

Answer 25

scarring is usually seen with conjunctival and cutaneous lesions (intraoral = rare scarring, may see intact blisters)

Question 26

Mucous Membrane Pemphigoid often presents as _______ gingivitis. What does this mean?

Answer 26

"desquamative gingivitis" - erythema, desquamation, ulceration - seen with several disorders, not just MMP

Question 27

What is the most significant aspect of MMP?

Answer 27

ocular involvement - scarring obstructs the orifices of glands that produce the tear film, resulting in dry eye - dryness leads to keratinization of the corneal epithelium, leading to blindness

Question 28

For histological purposes, where should the sample of MMP be taken from?

Answer 28

periphery of the lesion, including a generous portion of normal mucosa (epithelium shears off easily)

Question 29

What are the microscopic characteristics of MMP?

Answer 29

SUBepithelial cleft formation- separation of the epithelium from the connective tissue at the basement membrane - MMP: between epithelium and CT - PV: within epithelium

Question 30

Antibodies in PV will attack the _______; Antibodies in MMP will attack the ______.

Answer 30

PV= desmosomes MMP= hemidesmosomes (3 letters = longer word; 2 letters = shorter word)

Question 31

Oral mucosa submitted for MMP testing should be taken from _______cm away from the areas of ulceration. Tissue should, ideally, be submitted in both ______ solution and _____ solution.

Answer 31

0.5-1.0 cm Michel's formalin

Question 32

Linear deposition of immunoreactants at the BMZ, with a positive DIF and a negative IFF would indicate that the patient has _____.

Answer 32

mucous membrane pemphigoid | PV: both DIF and IFF are positve (MMP: only DIF is positive)

Question 33

What percentage of patients will have circulating autoantibodies against hemidesmosomes (MMP)?

Answer 33

5-25% of MMP patients will have circulating antibodies (which is why IFF is negative)

Question 34

How is MMPemphigoid treated?

Answer 34

Depends on extent of involvement: - frequent prophylaxis every 3-4 mos - oral lesions only = topical steroids, tetracycline/niacinamide or dapsone may be sufficient) - ocular involvement = systemic immunosuppressive therapy ***remember, PV = systemic only! topical has no effect***

Question 35

What is the prognosis of MMP?

Answer 35

rarely fatal, condition is usually controlled - blindness if ocular disease is left untreated - rarely resolves spontaneously

Question 36

True or False: Bullous pemphigoid usually affects a younger population.

Answer 36

False, older

Question 37

What is the most common initial complaint of bullous pemphigoid? Second most common initial complaint?

Answer 37

1. pruritus | 2. cutaneous blisters

Question 38

Which type of lesions are seen most commonly with bullous pemphigoid?

Answer 38

cutaneous | *oral is uncommon: 8-39%

Question 39

How often are oral lesions found with bullous pemphigoid?

Answer 39

8-39%

Question 40

True or False: BP Clefting of the epithelium is intraepithelial.

Answer 40

False, BP is suBBBBepithelial (like MMP)

Question 41

True or False: Both DIF and IFF are positive for bullous pemphigoid.

Answer 41

True

Question 42

True or False: Most BP cases resolve spontaneously.

Answer 42

True, most within 1-2 years | management is similar to MMP

Question 43

Erythema Multiforme is an ulcerative disorder that has _____ onset and is self-limiting within _____ weeks.

Answer 43

acute | 2-6 weeks

Question 44

Erythema Multiforme is more likely to affect which of the following: - 20 year old male - 20 year old female - 50 year old male - 50 year old female

Answer 44

20 year old female (as of recently. Used to believe that it affected males more, but it is probably immune-mediated so it makes sense that females are more likely)