1/
BULLOUS PEMPHIGOID, a #dermatology #tweetorial!
#MedEd #FOAMEd #medtwitter #dermtwitter #medthread pc:@dermnetnz
A patient with active bullous #pemphigoid comes to see you. What do you expect on your exam?
BULLOUS PEMPHIGOID, a #dermatology #tweetorial!
#MedEd #FOAMEd #medtwitter #dermtwitter #medthread pc:@dermnetnz
A patient with active bullous #pemphigoid comes to see you. What do you expect on your exam?
2/
#bullouspemphigoid (BP) is an autoimmune blistering disorder where the pt's immune system makes auto-antibodies targeting BPAg 1&2 (BP230/BP180). Since these Ags are in the hemidesmosome, the split is lower in the skin, making for tense blisters.
pc: sciencedirect.com/science/articl…
#bullouspemphigoid (BP) is an autoimmune blistering disorder where the pt's immune system makes auto-antibodies targeting BPAg 1&2 (BP230/BP180). Since these Ags are in the hemidesmosome, the split is lower in the skin, making for tense blisters.
pc: sciencedirect.com/science/articl…
3/
Clinically, tense blisters (as seen in BP) usually go with a negative nikolsky, whereas flaccid blisters (seen in pemphigus) would have a positive nikolsky.
An easy mnemonic is:
pemphiguS = Superficial
pemphigoiD = Deep
Clinically, tense blisters (as seen in BP) usually go with a negative nikolsky, whereas flaccid blisters (seen in pemphigus) would have a positive nikolsky.
An easy mnemonic is:
pemphiguS = Superficial
pemphigoiD = Deep
4/
Remember though that over time, all blisters will become flaccid, and will turn purulent. So when examining a patient, it's always important to look for an early lesion to help with the diagnosis.
Notice how in the pic, some of the bullae are either flaccid or just erosions!
Remember though that over time, all blisters will become flaccid, and will turn purulent. So when examining a patient, it's always important to look for an early lesion to help with the diagnosis.
Notice how in the pic, some of the bullae are either flaccid or just erosions!
5/
While we're on the exam, it's important to look at mucosa. While there is another dz called mucous membrane pemphigoid, BP can involve mucosa. This paper showed 17% of BP patients have mucosal involvement, and it's associated with worse prognosis!
jamanetwork.com/journals/jamad…
While we're on the exam, it's important to look at mucosa. While there is another dz called mucous membrane pemphigoid, BP can involve mucosa. This paper showed 17% of BP patients have mucosal involvement, and it's associated with worse prognosis!
jamanetwork.com/journals/jamad…
6/
Remember that erythema is harder to see on darker skin. Notice how it's hard to make out the pink hue underlying the bullae here?
pc: uptodate.com/contents/clini…
Remember that erythema is harder to see on darker skin. Notice how it's hard to make out the pink hue underlying the bullae here?
pc: uptodate.com/contents/clini…
7/
Diagnosing BP is usually straightforward. a skin biopsy for H+E & Direct immunofluorescence (DIF) is usually used.
The biopsy usually shows eosinophilic spongiosis, and a split at the Dermo-epidermal junction. The DIF would show linear deposition if IgG and C3 at the DEJ.
Diagnosing BP is usually straightforward. a skin biopsy for H+E & Direct immunofluorescence (DIF) is usually used.
The biopsy usually shows eosinophilic spongiosis, and a split at the Dermo-epidermal junction. The DIF would show linear deposition if IgG and C3 at the DEJ.
8/
Sometimes, we'll also send the ELISA tests to check BP antibody titers from blood. This is helpful when the patient might refuse biopsy but is okay with a blood draw, or if the DIF might be falsely negative. Sadly, the Sn/Sp is rather low at 66/89%.
link.springer.com/article/10.100…
Sometimes, we'll also send the ELISA tests to check BP antibody titers from blood. This is helpful when the patient might refuse biopsy but is okay with a blood draw, or if the DIF might be falsely negative. Sadly, the Sn/Sp is rather low at 66/89%.
link.springer.com/article/10.100…
9/
A DO-NOT-MISS opportunity is taking a good drug hx. Here's a little article we published showing that antibiotics and diuretics were the most commonly missed drugs that induced BP. Missing the drug trigger led to more immunosuppressants for those pts! jaad.org/article/S0190-…
A DO-NOT-MISS opportunity is taking a good drug hx. Here's a little article we published showing that antibiotics and diuretics were the most commonly missed drugs that induced BP. Missing the drug trigger led to more immunosuppressants for those pts! jaad.org/article/S0190-…
10/
So how might you treat BP? There's a whole ladder of therapy one can try. This is probably a good time to remind everyone to call their neighborhood #dermatologist for assistance!
So how might you treat BP? There's a whole ladder of therapy one can try. This is probably a good time to remind everyone to call their neighborhood #dermatologist for assistance!
11/
The non-immunosuppressive options include:
- topical steroids
- doxycycline + niacinamide
- dapsone
- IVIG
Immunosupressants:
- Prednisone
- Mycophenolate mofetil
- Aazathioprine
- Rituximab
Newer agents being reported:
- Dupilumab
- Omalizumab
I'm sure I missed a few...
The non-immunosuppressive options include:
- topical steroids
- doxycycline + niacinamide
- dapsone
- IVIG
Immunosupressants:
- Prednisone
- Mycophenolate mofetil
- Aazathioprine
- Rituximab
Newer agents being reported:
- Dupilumab
- Omalizumab
I'm sure I missed a few...
12/
RECAP!
- BP is an autoimmune disorder from antibodies targeting BPAG1&2 in the skin.
- Clinically see tense blisters, - nikolsky; mucosa can be involved.
- Take a drug history!
- Diagnose with biopsy or Ab titers from blood.
- Call a #dermatologist for help with treatment!
RECAP!
- BP is an autoimmune disorder from antibodies targeting BPAG1&2 in the skin.
- Clinically see tense blisters, - nikolsky; mucosa can be involved.
- Take a drug history!
- Diagnose with biopsy or Ab titers from blood.
- Call a #dermatologist for help with treatment!
13/
For anyone interested in reading about blistering diseases on a more general level, here's a #tweetorial I made almost a year ago looking at this group of diseases:
Hope this helps some folks out there! Until next time!
For anyone interested in reading about blistering diseases on a more general level, here's a #tweetorial I made almost a year ago looking at this group of diseases:
Hope this helps some folks out there! Until next time!
