Bullous Pemphigoid

00:02 Now, let's talk about the actual disease that our patient ultimately has; bullous pemphigoid. We've talked a little bit about it already, but let's just highlight a few key points. It is, like pemphigus vulgaris, an autoimmune bullous disease. Most common in men and women over the age of 60. And in this case, the antibodies are directed against proteins at the epithelial basement membrane. Again, at that dermal-epidermal junction.

00:28 Regions are often pruritic, initially, urticarial plaques, and ultimately, over time, you'll start to see these tense bullae appear on normal or on erythematous skin, and it can take months for those things to develop.

00:42 These patients will have a negative Nikolsky sign.

00:45 Rubbing on the skin will not lead the skin to slough off.

00:49 Patients don't have constitutional symptoms, as was the case in our patient.

00:53 Very infrequently will you see the mucosal tissues involved, and if you were to perform a biopsy, you would see immunofluorescence with linear IgG or C3 staining along that basement membrane, at that junction we talked about.

01:08 Treatment is going to be topical or, more likely, oral glucocorticoids, which you may need to prescribe for a number of months.

01:15 And again, you're going to use those steroid-sparing agents as soon as you can with azathioprine or micophenolate mofetil.