Vesiculobullous Disease Basic Science 1

Question 1

Name the cadherins.

Answer 1

Calcium dependent
Classic-E, P, & N-cadherin
Desmosomal-Desmoglein, Desmocollin

Question 2

Name the armadillo family proteins

Answer 2

Plakoglobin, plakophilin, β-catenin

Question 3

Name the plakins and what is there function?

Answer 3

adhesion of keratin intermediate filaments

Desmoplakin, BPAg1, plectin, envoplakin, periplakin

Question 4

The lamina densa is composed mainly of what type of collagen?

Answer 4

Type IV

Question 5

The anchoring fibrils contain what type of collagen?

Answer 5

Type VII-Bullous SLE, EBA, DEB

Question 6

What creates the negative charge in the lamina densa?

Answer 6

Perlecan-heparin sulfate progeoglycan

Question 7

What tissue do you use for indirect immunoflourescence for pemphigus vulgaris? paraneoplastic pemphigus? Pemphigus foliaceus?

Answer 7

PV-Monkey’s are vulgar, PNP-Rat’s I have cancer, PF-Guinea pigs eat foliage

Question 8

What is Nikolski’s sign?

Answer 8

slight pressure, twisting, or rubbing on skin shears epidermis, reveals dermis

Question 9

What is Asboe-Hansen’s sign?

Answer 9

“bulla spreading phenomenon” blisters spread laterally w/ pressure on surface of blister

Question 10

What is the antibody present in the mucosal variant of pemphigus vulgaris?

Answer 10

Desmoglein 3 (130 kD)

Question 11

What antibody is present in mucocutatneous pemphigus vulgaris?

Answer 11

Desmoglein 1 (160kD) & 3 (130kD)

Question 12

What HLA is the most common for pemphigus vulgaris?

Answer 12

DR4, DR6

Question 13

What is the standard histopathology for pemphigus vulgaris?

Answer 13

Suprabasilar acantholysis with tombstoning involving hair follicle and sebaceous glands (differentiates from Hailey-Hailey)

Question 14

What antibody is present in pemphigus foliaceus?

Answer 14

Desmoglein 1 (160kD)

Question 15

Pemphigus foliaceus is more common than PV in what countries?

Answer 15

Finland, Tunisia, Brazil (everywhere else PV is much more common than PF)

Question 16

What is the histology of pemphigus foliaceus?

Answer 16

Subcorneal /intragranular acantholysis with “cling ons”

Question 17

What arthropod causes Fogo Sevalgem (endemic PF)?

Answer 17

Simulium nigrimanum-also causes onchocerciasis

Question 18

What antibody do you have in pemphigus erythematosus?

Answer 18

anti desmoglein 1

Question 19

What antibodies are present in paraneoplastic pemphigus?

Answer 19

everything but BPAg2

Question 20

What is the most common malignancy associated with paraneoplastic pemphigus?

Answer 20

non-Hodgkins lymphoma (40%), CLL (30%), Castleman’s (10%-most common in children), Thymoma, Sarcoma & Waldenstrom’s macroglobulinemia (all 6%)

Question 21

Ddx for subepidermal blister + neutrophils predominantly?

Answer 21

Herpetic LIPS
Herpetic-DH
L = lupus (bullous)
I = linear IgA
P = pemphigoid (BP)
S = Sweet's syndrome

Question 22

What is the DIF pattern in pemphigus vulgaris?

Answer 22

Chicken wire with IgG4

Question 23

What are the two subtypes of pemphigus vegetans?

Answer 23

Neuman (MC and severe), and Hallopeau (mild, pustular)

Question 24

Name the drugs implicated in pemphigus foliaceus?

Answer 24

Same as PV-Thiol drugs (captopril, penicillamine, gold thiosulfate), sulfa-releasing, penacillamine, nifedipine

Question 25

What is the HLA associated with paraneoplastic pemphigus?

Answer 25

HLA-DRB1

Question 26

What is the most consistent sign in paraneoplastic pemphigus?

Answer 26

intractable stomatitis

Question 27

What process is associated with a poor prognosis in paraneoplastic pemphigus?

Answer 27

bronchiolitis obliterans

Question 28

Where does the IIF focus to in BP?

Answer 28

Roof

Question 29

What lab abnormality can be associated with impetigo herpetiformis?

Answer 29

hypocalcemia (hypoparathyroidism)

Question 30

What is the drug of choice for cicatricial pemphigoid with rapidly progressive eye disease?

Answer 30

cyclophosphamide