Vesiculobullous Diseases: Bullous Pemphigoid

Question 1

Bullous Pemphigoid

Clinical Feature. Lesion & Symp

Answer 1

• chronic autoimmune bullous eruption characterized by pruritic, tense, subepidermal bullae on an
erythematous or normal skin base
• can present as urticarial plaques without bullae

Question 2

Bullous Pemphigoid

common sites:

Answer 2

flexor aspect of forearms, axillae, medial thighs, groin, abdomen, mouth in 33%

Question 3

Bullous Pemphigoid

Pathophysiology

Answer 3

IgG produced against dermal-epidermal basement membrane proteins (hemidesmosomes) leads to
subepidermal bullae

Question 4

Bullous Pemphigoid
Epidemiology.
Investigations

Answer 4

mean age of onset: 60-80 yr old, F=M

Question 5

Bullous Pemphigoid

Investigations

Answer 5

• immunofluorescence shows linear deposition of IgG and C3 along the basement membrane
• anti-basement membrane antibody (IgG) (pemphigoid antibody detectable in serum)

Question 6

Bullous Pemphigoid

Prognosis. Fatality

Answer 6

• heals without scarring, usually chronic

* rarely fatal

Question 7

Bullous Pemphigoid

Management. Topical

Answer 7

topical potent steroids (clobetasol) may be as effective as systemic steroids in limited disease

Question 8

Bullous Pemphigoid

Management. PO

Answer 8

prednisone 0.5-1 mg/kg/d until clear, then taper ± steroid-sparing agents (e.g. azathioprine,
cyclosporine, mycophenolate mofetil)

Question 9

Bullous Pemphigoid

Management. PO alternatives

Answer 9

tetracycline ± nicotinamide is effective for some cases

• immunosuppressants such as azathioprine, mycophenolate mofetil, cyclosporine

Question 10

Bullous Pemphigoid

Management. Systemic Alternatives

Answer 10

IVIg and plasmapheresis for refractory cases