Pemphigoid-like disease

Question 1

Epidermolysis bullosa acquisita pathophysiology

Answer 1

IgG autoantibodies to NC1 terminal of type VII collagen in basement membrane zone
- blisters form in sublamina densa

Question 2

EBA cliniacl presentation

Answer 2

Chronic, two distinct presentations, characterized by skin fragility and trauma induced blisters that heal with scarring and milia

Question 3

Classic EBA presentation

Answer 3

• tense blisters on non-inflammatory base on trauma prone areas (palms, soles, etc)
• lesions heal with scaring and milia (can look like PCT)
• can also affect mucous membranes looking like cicatricial pemphigoid

Question 4

BP like EBA

Answer 4

• inflammatory type (aka blisters on inflammatory base)
• widely distributed on trunk and flexural surfaces ‘
• pruritus and minimal skin fragility
• some lesions heal w/o scaring and milia

Question 5

EBA diagnosis

Answer 5

Need to do salt-split skin (will see IgG deposits on dermal side unlike in BP where it will be epidermal side)

Question 6

Associated comorbidities in EBA

Answer 6

25% of EBA patient’s have Crohn’s disease

Question 7

Tx of EBA

Answer 7

poor response to tx in general, can try high dose steroids, dapsone, imuran, colchicine, cyclosporine, rituximab, and IVIG