Bullous Pemphigoid

Question 1

What is the pathophysiology and aetiology (cause) of Bullous Pemphigoid?

Answer 1

Autoimmune, SUP EPIDERMAL blistering due to IgG autoantibodies for the basement membrane protein BP1 and BP2.
IgG binds to basement membrane hemidesmosomes and activates the inflammatory cascade. Separation of hemidesmosomes gives the bullae.

• elderly >65yrs
• vaccinations (in children with condition affecting face, palms and soles)
• NSAIDs, furosemide, antibiotics
• UV radiation / x-ray

Question 2

Signs and symptoms of Bullous Pemphigoid?

Answer 2

• thick tense blister and erosions appear typically in flexural regions of limb and trunk
• present acutely or insidiously
• often a pruritic prodromal rash and region
• self limiting at 2 years
• usually heal without scarring (unless cicatricial pemphigoid involving the mucous membrane)

(no oral and no nikolsky sign - rubbing)

Question 3

Photos of Bullous Pemphigoid

Answer 3

Yeah, put some in

Question 4

Management of Bullous Pemphigoid?

Answer 4

• doxycycline is used for its anti-inflammatory effects!
• corticosteroids (prednisolone)
• immunosuppressants (azathioprine / methotrexate)
• topical if localised (vpotent Dermovate), systemic if severe (pred then reduce)

Wean of meds until back to normal and remission.