Vesiculobullous Disorders

Question 1

What type of disease is bullous pemphigoid?

Answer 1

autoimmune disease, causing the skin to cause blisters

Question 2

Name the three layers of the skin

Answer 2

epidermis, dermis, hypodermis

Question 3

What type of cell predominantly makes up the epidermis?

Answer 3

keratinocytes

Question 4

What is the bottom layer of the epidermis called?

Answer 4

stratum basale

Question 5

What is the function of the stratum basale?

Answer 5

made up of stem cells (basal cells) that continually divide to form new keratinocytes

Question 6

Where is the basement membrane found?

Answer 6

below the epidermis

Question 7

What are hemidesmosomes?

Answer 7

protein complex found at the bottom of the basement membrane, that holds basal cells in place

Question 8

List some drugs that may trigger bullous pemphigoid in a person with a genetic predisposition

Answer 8

furosemide
captopril
penicillamine
NSAIDs
antibiotics

Question 9

What type of immune reaction is seen in bullous pemphigoid?

Answer 9

type II hypersensitivity reaction

Question 10

Describe the immune reaction that causes bullous pemphigoid

Answer 10

antigen-binding region of IgG binds to hemidesmosomes
antibodies also activate complement system

Question 11

What type of blisters are seen in bullous pemphigoid?

Answer 11

subepidermal bullae

Question 12

Where is bullous pemphigoid most commonly located?

Answer 12

lower abdomen, flexor side of forearms, anterior/inner thighs

Question 13

Describe the progression of blisters in bullous pemphigoid

Answer 13

red itchy rash develops into large bullae
these leave behind crusted lesions that heal without scarring

Question 14

What is the early presentation of bullous pemphigoid?

Answer 14

red, itchy rash

Question 15

What is the Nikolsky’s sign?

Answer 15

lateral pressure causes split between layers of the epidermis

Question 16

In what condition is Nikolsky’s sign present?

Answer 16

pemphigus vulgaris, NOT bullous pemphigoid

Question 17

What investigations can be done to confirm bullous pemphigoid?

Answer 17

skin biopsy to look for antibodies and complement infiltration
blood tests for auto-antibodies

Question 18

What is the management for bullous pemphigoid?

Answer 18

stop any medications that could be triggering it
corticosteroids

Question 19

What type of disease is pemphigus vulgaris?

Answer 19

rare autoimmune skin disorder, causing blisters

Question 20

What are desmosomes?

Answer 20

protein complexes that hold together cells of the epidermis/keratinocytes

Question 21

What is mucosa?

Answer 21

lining of the inside of the body

Question 22

What attaches mucosal cells to one another?

Answer 22

desmosomes

Question 23

What type of immune reaction is seen in pemphigus vulgaris?

Answer 23

type II hypersensitivity reaction

Question 24

What can trigger pemphigus vulgaris in somebody who has a genetic predisposition?

Answer 24

herpes virus infection
drugs e.g., captopril or certain antibiotics

Question 25

What is mucocutaneous pemphigus vulgaris?

Answer 25

where the disease affects both skin and the mucosa

Question 26

What is mucosal pemphigus vulgaris?

Answer 26

where the disease affects just the mucosa, not the skin

Question 27

Describe the immune reaction that causes pemphigus vulgaris

Answer 27

IgG binds to desmogleins, triggering apoptosis proteases then get released that break down desmosomes

Question 28

What is caused by the break down of desmosomes in pemphigus vulgaris

Answer 28

acantholysis - the process of cells letting go of one another this is what forms blisters

Question 29

What is meant by the term 'tombstoning'?

Answer 29

basal cells remain anchored to the basement membrane, but separate from other cells of the epidermis

Question 30

What type of blisters are seen in pemphigus vulgaris?

Answer 30

intraepidermal bullae

Question 31

Describe the early presentation of pemphigus vulgaris

Answer 31

bullae and ulcerations on the oral mucosa leads to difficulty eating and drinking mucocutaneous form also develops macules

Question 32

Where is pemphigus vulgaris most commonly located on the body?

Answer 32

can be anywhere, usually with mucosal involvement

Question 33

Describe the progression of blisters in pemphigus vulgaris

Answer 33

red macules turn into bullae, that then rupture and leave painful erosions

Question 34

What investigations can be used to confirm pemphigus vulgaris?

Answer 34

skin biopsy to look for acantholysis and tombstoning

Question 35

What is the management for pemphigus vulgaris?

Answer 35

topical/systemic corticosteroids, depending on severity rituximab in some cases topical rinses e.g., viscous lidocaine to reduce pain