Pemphigus, Pemphigoid, & Rarer Conditions

Question 1

What is Stevens-Johnson syndrome + 2 specific features?

Answer 1

Extreme form of erythema multiforme with bullae and systemic involvement including the lungs and kidneys

• gives classical “target lesions”
• extremely acute onset

Question 2

What is toxic epidermal necrolysis?

Answer 2

Rapid widespread loss of large areas of the epidermis

Question 3

What are Stevens-Johnson syndrome and toxic epidermal necrolysis most commonly secondary to?

Answer 3

Drug reactions - check Hx for recent drug introductions/changes

Question 4

Presentation of pemphigus (pemphigus vulgaris)?

Answer 4

Flaccid blisters
Erosion in erythematous patches
- these burst very easily and heal very slowly
Nikolsky sign - skin of the blister can be made to slide off with the push of a finger
Oral involvement is common

Question 5

Presentation of pemphigoid (bullous pemphigoid)?

Answer 5

Tense blisters

Difficult to break

Question 6

What are the similarities and distinguishing features of pemphigus and pemphigoid?

Answer 6

Both are autoimmune disorders
Distinction is the site of skin splitting
- Pemphigus - within the epidermis
- Pemphigoid - at the demo-epidermal junction

pemphiguS - Superficial
pemphigoiD - Deep

Question 7

Treatment of pemphigoid?

Answer 7

Moderate dose oral corticosteroids

Steroid-sparing immunosuppressants e.g. azathioprine