This is an autoimmune blistering disorder that can be triggered by drugs!
Hey #medtwitter, what do you think is one of common culprits for causing this eruption?
2/ You'd be correct if you said vancomycin!
Vanco triggers IgA to attack proteins in the hemidesmosome that holds epidermis to the basement membrane. That means this is a part of the PEMPHIGOID group.
So you get TENSE blisters with a NEGATIVE NIKOLSKY.
3/ For the #dermatology residents who need to memorize this, remember that the antigen that is targeted is the 97 kDa portion of the extracellular domain found in BPAg2.
For everyone, remember this is part of the pemphigoiD (D for deep) group, hence the exam findings.
4/ On clinical exam, the unusual thing about this blistering eruption is the configuration of the bullae. They are usually in an ANNULAR pattern!
So you might ask, wait - why is it called LINEAR IgA and not Annular IgA?!
5/ It's got to do with the direct immunofluorescence pattern. On the DIF, you see a linear deposition of IgA at the Basement Membrane Zone, leading to the name (as opposed to IgG in bullous pemphigoid).
But clinically, again, you see annular tense bullae with negative Nikolsky.
6/ While Vancomycin is the most common trigger, it's important to know other drugs can do it too (NSAIDs, ACEi, Diuretics, Abx, etc).
Additionally, kids can get an idiopathic version! Same clinical and pathologic findings, but we call it Chronic Bullous Disease of Childhood.
7/ This tweet goes out to the derm residents out there. Remember that when you see IgA in the pathophysiologic process, you should think of neutrophils. That's the case here too!
So treatment is d/c of offending trigger, and starting an anti-PMN drug like dapsone or colchicine.
8/ If a recent drug doesn't seem to be at fault, there are other associated conditions (eg: IBD, cancer). I've seen IBD drive Linear IgA, in which case co managing with GI is super important. That's where choosing something that works on both gut and skin is critical!
9/ Let's summarize!
✅Linear IgA is a blistering disease where IgA targets the BMZ, causing annular tense bullae.
✅Vancomycin (and other drugs can trigger this) - as can IBD and cancer. Also can see in kids!
✅Treatment is addressing the underlying trigger, and anti-PMN drugs.
10/10
Thanks for joining for this short #thread/#tweetorial on Linear IgA! It's relatively rare, but a good one to know, especially if you ever reach for vancomycin in your patients!
Leave a suggestion for another topic if you'd like. Thanks everyone!
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2/ The "pemphigus" part of the name means we are similarly dealing with an EPIDERMAL blistering disease, much like it's better known cousin, "pemphigus vulgaris" (PV).
If you haven't had a chance yet, take a look at my prior #tweetorial on PV:
3/ Before we get into the nitty gritty details of PF vs PV, a reminder that in pemphiguS, we're dealing with a SUPERFICIAL desmosome antigen target, so compared with pemphigoiD (that's DEEP), you're still going to get the + nikolsky, flaccid bullae, etc.
2/ If you answered, "hands, feet, and ears," you're correct! This is tricky, and it wasn’t until dermatology residency that I learned it’s not palms and soles!
If you haven't yet, take a look at my old #tweetorial on acral rashes as a primer.
What color do you expect to see when you hear NXG?
2/ Yes, yellow! Whenever you hear something is “xanthomatous,” expect to see something yellow on exam! Kudos to all of you who guessed some form of a xanthomatous process on our prior mystery diagnosis tweet!👇
3/ This diagnosis occurs classically by the eyes and correspondingly can cause ophthalmologic issues, so for those of you who suggested a referral to ophtho, absolutely agree!
Let's start ourselves off with a question: Which one of the following conditions will lead to scarring?
2/ The correct answer is Pyoderma Gangrenosum! This illustrates a quick first point - scarring only occurs if you damage the skin into dermis and beyond. Epidermal damage heals without scarring, which is why the first 3 don't lead to scarring!
3/ So what exactly is a scar?
Scarring is a normal part of healing that at its root, is extra collagen laid down to repair skin injury.
However, sometimes the process gets out of hand and exuberant which leads to hypertrophic scars (pic 1) keloids (pic 2)!