Vesiculobullous Disease Basic Science 2

Question 1

What are the two type of IgA pemphigus?

Answer 1

Subcorneal pustulosis (desmocollin 1), intraepidermal neutrophyilic like (Desmoglein 1 & 3)

Question 2

What HLA is associated with bullous pemphigoid?

Answer 2

HLA-DQB1*0301

Question 3

What percentage of patients have oral involvement in bullous pemphigoid?

Answer 3

20%

Question 4

What does DIF show in bullous pemphigoid?

Answer 4

C3 & IgG linear deposition on the basement membrane

Question 5

Which antibodies will fix complement in vitro pemphigoid or pemphigus?

Answer 5

Pemphigoid

Question 6

What is a clinical feature to help distinguish between pemphigoid gestationis and PUPPP?

Answer 6

Pemphigoid gestationis involves the periumbilical area it is spared in PUPPP

Question 7

What does the DIF look like in pemphigoid gestationis? for PUPPP?

Answer 7

C3 (100%) and IgG (25-40%) linear along basement membrane. PUPPP is negative

Question 8

Patients who have pemphigoid gestationis are at increased risk for what disease?

Answer 8

Graves disease

Question 9

Are there any risks to the fetus in pemphigoid gestationis?

Answer 9

small for gestational age and prematurity

Question 10

What is the typical presentation of PUPPP?

Answer 10

Third trimester in nulliparous/primagravid urticaria in abdominal striae and periumbilical sparing

Question 11

What is the most common presenting symptom in cicatricial pemphigoid?

Answer 11

desquamative gingivitis (70%)

Question 12

What is the cicatricial pemphigoid variant that has cutaneous head and neck involvement and no mucosal involvement?

Answer 12

Brunsting-Parry

Question 13

What antibody is associated with mucosal and skin cicatricial pemphigoid?

Answer 13

BPAg2 (Distal C terminal)

Question 14

What antibody is associated with ocular cicatricial pemphigoid?

Answer 14

Beta 4 integrin (uncein)

Question 15

What antibody is associated with malignancy in cicatricial pemphigoid?

Answer 15

laminin 5 (epiligrin)

Question 16

What is the antibody associated with epidermal bullose acquisita (EBA)?

Answer 16

Collagen VII (NC1 domain)

Question 17

Epidermal bullosa acquisita (EBA) is most commonly associated with what other disease?

Answer 17

inflammatory bowel disease specifically crohn’s

Question 18

What is another name for dermatitis herpetiformis?

Answer 18

Duhring’s disease

Question 19

Dermatitis herpetiformis is associated with what HLA?

Answer 19

HLA-B8 positive, HLA-DQ2, DQ8

Question 20

What does the DIF look like in dermatitis herpetiformis?

Answer 20

granular deposition of IgA along the DE junction

Question 21

What antibodies do you see in dermatitis herpetiformis?

Answer 21

Antiendomysial antibodies
Antiepidermal transglutaminase antibodies
Antigliadin antibodies

Question 22

What is a common drug to cause linear IgA?

Answer 22

Vancomycin

Question 23

A rim of blisters around an erosion is common for what disease?

Answer 23

Linear IgA (Crown of jewels)

Question 24

Linear IgA is associated with what antibody?

Answer 24

BPAg2 (97-120 kD epitope)

Question 25

Cicatricial pemphigoid that is associated with what type of malignancy?

Answer 25

Adenocarcinoma

Question 26

What is another name for pemphigus erythematosus?

Answer 26

Senear-Usher syndrome

Question 27

List the autoantibodies and their weights?

Answer 27

``` 500 kD – Plectin 250 kD – Desmoplakin 1 230 kD – BPAg1 210 kD – Desmoplakin II 190 kD – Periplakin 160 kD – Desmoglein 1 130 kD – Desmoglein 3 Desmocollins 1+2 ```