vesiculobullous disease and blistering oral disease

Question 1

What is pemphigoid?

Answer 1

Thick walled blisters - clear or blood filled
Exposed connective tissue when blisters burst
Loss of epithelial barrier leads to dehydration

Question 2

What are the different types of pemphigoid?

Answer 2

Bullous pemphigoid - affects skin
Mucous membrane pemphigoid - all mucous membranes, eye, genital, oral
Cicatritial pemphigoid - mucousal with scarring

Question 3

Describe the histopathology of pemphigoid

Answer 3

Sub epithelial split
Antibodies attack the hemidesmosomes at the basement membranes that attach the epithelium to the connective tissue

Question 4

How does direct immunofluorescence work for pemphigoid?

Answer 4

Linear staining seen along basement membrane
C3 and IgG antibodies detected in this area
IgA occasionally found

Question 5

How is pemphigoid managed?

Answer 5

Steroids
Immune modulating drugs

Question 6

What is pemphigus?

Answer 6

Immune mediated antibody disease causing intra epithelial bullae
Found in skin and mucosa

Question 7

Describe the histopathology of pemphigus

Answer 7

Antibodies attack the desmosomes joining epithelial cells, forming intraepithelial bullae
See loss of epithelium
Basal cells still attached to basement membrane
Tzank cells seen

Question 8

How does direct immunofluorescence work in pemphigus

Answer 8

Antibodies binding all around cells as desmosomes attach epithelial cells to each other
Basket-weave pattern
C3 and IgG antibodies

Question 9

What is the commonest type of pemphigus?

Answer 9

Pemphigus vulgaris

Question 10

What is the main clinical difference between pemphigus and pemphigoid?

Answer 10

In pemphigus you rarely see intact bullae, you see erosions instead

Question 11

What should you do when examining salivary glands?

Answer 11

E/O - feel major salivary glands
I/O - look at minor salivary glands
- duct orifices
- fluid expression

Question 12

What are the functions of saliva?

Answer 12

Acid buffering
Mucosal lubrication - speech and swallowing
Taste facilitation
Antibacterial - prevents oral infections

Question 13

Give 4 causes of a dry mouth?

Answer 13

Salivary gland disease
Drugs - reduce stimulation of glands to produce saliva
Medical conditions and dehydration
Anxiety and somatisation disorders

Question 14

How does salivary flow change with age?

Answer 14

Loss of acinar tissue in the glands

Question 15

Give 4 examples of antimuscarinic drugs

Answer 15

Tricyclic antidepressants
Antipsychotics
Antihistamine
Diuretics

Question 16

Name 4 conditions which cause direct salivary gland problems?

Answer 16

Aplasia
Sarcoidosis
HIV
Gland infiltration
Cystic fibrosis

Question 17

What does ectodermal dysplasia affect?

Answer 17

Affects hair, nails, teeth, salivary glands and sweat glands
May affect hearing and vision

Question 18

What is sarcoidosis?

Answer 18

Multi system disease often seen as granulomatous change
Affects skin and saliva

Question 19

How does HIV affect saliva?

Answer 19

Can cause lymphal proliferative change in the salivary glands
If increase in size of salivary gland, patient should be offered a HIV test

Question 20

How does cancer treatment affect saliva?

Answer 20

Radiotherapy causes loss of vascular supply
Drugs can accumulate and kill off acinar cells

Question 21

Describe the Challacombe scale of mucosal dryness

Answer 21

Assesses degree of mucosal dryness from 1-10

Question 22

What are the investigations for salivary disease?

Answer 22

Blood tests - FBC, U&E, anti Ro antibody, anti La testing
Salivary flow assay
Labial gland biopsy
Imaging - ultrasound, radiographs, sialography