Blistering Skin Disease

Question 1

Give examples of common causes of skin blisters

Answer 1

Infection - HSV, VSV, impetigo, tines pedis, cellulitis
Insect bites
Trauma - burns and friction
Eczema

Question 2

Give examples of immunobullous diseases

Answer 2

Dermatitis herpetiformis
Phemigus
Bullous phemigoid

Question 3

What is the name for mechanobullous disease?

Answer 3

Epidermyloysis bullosa

Question 4

What is epidermyloysis bullosa?

Answer 4

A spectrum of mechanobullous disease encompassing >30 inherited conditions in which there is mechanical fragility of the skin resulting in blistering

Question 5

What are the typical features of mechanobullous disease?

Answer 5

Mechanical fragility of skin
Recurrent blisters / erosions
Poor wound healing
Dystrophic /absent nails
Milia (tiny white bumps)
Atrophic scarring
Herpetiform blistering
Reticulate hyperpigmentation
Exuberant granulation tissue

Question 6

What investigations should be used for mechanobullous disease?

Answer 6

Immunofluorescence antigen mapping
Transmission electron microscopy
DNA mutation analysis

Question 7

How is mechanobullous disease managed?

Answer 7

Wound care
Treatment of superimposed infection
Nutritional supplementation
Lifestyle advice (avoiding skin trauma)

Question 8

At what age does mechanobullous disease typically present?

Answer 8

Birth or early infancy

Question 9

What are the potential complications of mechanobullous disease?

Answer 9

Failure to thrive
Growth retardation
Anaemia
Depression
Deformities of the hands and feet
Squamous cell carcinoma

Question 10

What is pemphigus?

Answer 10

A group of autoimmune diseases characterised by blistering of the epidermis and/or mucosa

Question 11

List the three subtypes of pemphigus in order of commonality (most common to least common)

Answer 11

Most common - pemphigus vulgaris
Middle - pemphigus follaceus
Least common - paraneoplastic pemphigus

Question 12

Which malignancies is paraneoplastic pemphigus associated with?

Answer 12

Non-Hodgkin’s lymphoma
CLL
Thymoma

Question 13

Describe the pathophysiology of pemphigus vulgaris and pemphigus follaceus?

Answer 13

IgG autoantibodies are directed against the desmosome resulting in blistering disease

Question 14

Describe the pathophysiology of paraneoplastic pemphigus?

Answer 14

IgG antibodies and cell mediated destruction of hemidesmosomes

Question 15

What investigations are used in pemphigus?

Answer 15

Histopathology - haemotoxylin/eosin staining
Direct and indirect immunofluoresence
ELISA looking for IgG autoantibodies
Endoscopy, CXR and CT chest in some cases to evaluate the extent of the disease

Question 16

Describe the presentation of pemphigus vulgaris and pemphigus follaceus?

Answer 16

Chronic pruritic and/or painful erosions or superficial blisters which typically affect the oral mucosa and skin in pemphigus vulgaris and which only affect the skin (typically face, scalp and upper trunk) in pemphigus follaceus

Question 17

Describe the management of pemphigus?

Answer 17

Wound care
Supportive care (analgesia, antiseptic baths, dental care, nutritional support)
Topical or oral corticosteroids
Biologics (Rituximab)
Oncology referral for paraneoplastic pemphigus

Question 18

What is bullous pemphigoid?

Answer 18

A chronic acquired autoimmune disease which results in sub-epidermal blistering

Question 19

What are the typical symptoms of the pre-bulbous prodromal stage of bullous pemphigoid?

Answer 19

Pruritus
Eczematous / urticarial lesions
Lasts weeks to months

Question 20

Describe the typical appearance of bullous pemphigoid?

Answer 20

Tense vesicles and bullae typically appearing on the flexor surfaces of the limbs, axillae, groin and abdomen

When blisters burst, there may be crusty eroded lesions which heal with hyperpigmentation

Question 21

What investigations are used for bullous pemphigoid?

Answer 21

Histopathology
Light microscopy
Direct and indirect immunofluorescence
ELISA

Question 22

How is bullous pemphigoid managed?

Answer 22

Topical (or oral) corticosteroids
Tacrlimus
Antihistamines
Steroid sparing agents - Ciclosporin or dapsone in some cases
Topical antibiotics as required

Question 23

What is dermatitis herpetiformis?

Answer 23

A rare blistering disease associated with coeliac disease

Question 24

Describe the appearance of dermatitis herpetiformis?

Answer 24

Small, pruritic blisters and papules on the elbows, extensor surfaces of the forearms, scalp and buttock

Lesions may become crusted and eroded when scratched

Question 25

What is the definitive management of dermatitis herptiformis?

Answer 25

Avoid gluten

Question 26

How can the rash of dermatitis herpetiformis be managed?

Answer 26

Dapsone

Sulphonamides