bullous pemphigoid / pemphigus vulgaris

Question 1

Bullous pemphigoid

Answer 1

development of antibodies against hemidesmosomal proteins BP180 and BP230

Question 2

features of bullous pemphigoid?

Answer 2

itchy, tense blisters typically around flexures
the blisters usually heal without scarring
there is stereotypically no mucosal involvement (i.e. the mouth is spared)
in reality around 10-50% of patients have a degree of mucosal involvement

Question 3

diagnosis of bullous pemphigoid?

Answer 3

immunofluorescence shows IgG and C3 at the dermoepidermal junction

Question 4

tx of bullous pemphigoid?

Answer 4

oral corticosteroids are the mainstay of treatment

n addition to a steroid-sparing immunomodulatory agent such as dapsone, mycophenolate mofetil, azathioprine, methotrexate or chlorambucil.

If you can’t take can’t corticosteroids or other immunomodulatory agents, your healthcare provider may prescribe oral tetracycline or doxycycline pills.

For refractory cases of bullous pemphigoid, your healthcare provider may prescribe rituximab or IVIG infusions.

Question 5

cause of Pemphigus vulgaris

Answer 5

antibodies directed against desmoglein 3, a cadherin-type epithelial cell adhesion molecule.

Question 6

Pemphigus vulgaris more common in ?

Answer 6

Ashkenazi Jewish population

Question 7

features of Pemphigus vulgaris

Answer 7

mucosal ulceration
Oral involvement is seen in 50-70%

skin blistering - flaccid, easily ruptured vesicles and bullae

typically painful but not itchy.

These may develop months after the initial mucosal symptoms.

Nikolsky’s describes the spread of bullae following application of horizontal, tangential pressure to the skin

Question 8

diagnosis of of Pemphigus vulgaris

Answer 8

acantholysis on biopsy

Question 9

tx of Pemphigus vulgaris

Answer 9

Corticosteroids: Medication to reduce inflammation (swelling), delivered by mouth, by injection (a shot) or topically (ointments or creams).

Immunosuppressive drugs: azathioprine or mycophenalate mofetil

Rituximab: A monoclonal antibody that targets problematic B cells.

Intravenous immunoglobulin:

Antibiotics: In some cases, infections can develop in pemphigus blisters. If this happens, your doctor may prescribe antibiotic medicine to treat the infection.