Vesiculobullous disorders

Question 1

What is a vesicle?

Answer 1

Small fluid lesion/blister which can rupture to form an ulcer

Question 2

What is a bullae?

Answer 2

Large blister

Question 3

What are intra epithelial vesicles?

Answer 3

Lesions formed within the epithelium as there is loss of cell to cell adhesion by breakdown of specialised attachment (desmosomes) = acantholysis

Question 4

What are sub epithelial vesicles?

Answer 4

Lesions are formed between the epithelium and the underlying lamina propria

Question 5

What is the aetiology of pemphigus vulgaris?

Answer 5

Most common in >65s
Mostly affects skin and mouth
Can be potentially fatal
Genetic - Ashkenazi jews

Question 6

What are the clinical features of pemphigus vulgaris?

Answer 6

Clear fluid filled blisters (bullae) that readily burst to form large irregular ulcers that spread.
Commonly affects buccal mucosa, palate and tongue
Fatal disease without whole body treatment

Question 7

What is the diagnostic features of pemphigus vulgaris?

Answer 7

Positive Nicolsky’s sign
Supra basal split with tzank cells in biopsy
Basket weave pattern on direct IF
Auto antibodies to Desmogen factor with indirect IF

Question 8

How is pemphigus managed?

Answer 8

Immunosuppressants such as azathioprine or methotrexate

Question 9

What is the aetiology of pemphigoid?

Answer 9

F>M >60yrs affected
Affects any part of mucosa
Periods of inactivity

Question 10

What are the clinical features of pemphigoid?

Answer 10

Thick walled blisters affecting full epidermis layer which are blood filled intact vesicles
Ulceration that leads to scarring
Can affect larynx and eyes

Can present with desquamative gingivitis

Question 11

What are the diagnostic features of pemphigoid?

Answer 11

Autoantibodies against basement membrane to form sub epithelial vesicles
Sub basal split on biopsy

Question 12

What is the Management of pemphigoid?

Answer 12

Analgesics, topical/systemic corticosteroids

Immunosuppressants

Question 13

What is the aetiology of erythema multiforme?

Answer 13

Acute inflammatory disease of skin and mucous membrane
Common in young adult males
Severe in Stevens Johnson syndrome

Question 14

What are the predisposing factors for erythema multiforme?

Answer 14

Infections - herpes

Drugs - antibiotics, anticonvulsants, allopurinol and NSAIDs

Question 15

What are the clinical features of erythema multiforme?

Answer 15

Vesicles and bullae which rupture to form ulcers and erosions
Common in lips, BM, palate, tongue and face
Target lesions or erythema of skin
Crusting of lips

Question 16

How is erythema multiforme managed?

Answer 16

Self limiting but will need supportive therapy such as fluids, soft diet and analgesics
Topical/systemic steroids

Question 17

What is the aetiology of epidermolysis bullosa?

Answer 17

Genetic or acquired condition

Appears shortly after birth affecting skin and mucous membranes

Question 18

What are the clinical features of epidermolysis bullosa?

Answer 18

Trauma to mucosa results in bullae formation
Healing leads to scaring making eating, swallowing speaking difficult
Symptoms may be mild

Question 19

What are other types of sub epithelial vesiculobullous disorders affecting oral mucosa?

Answer 19

Linear IgA disease
Dermatitis herpetiform
Bullous lichen planus
Bullous systemic lupus erythematous