Disease Severity and Treatment Setting of GPP

Aaron Farberg, MD, and Maria Lopes, MD, MS, discuss what makes generalized pustular psoriasis a potentially life-threatening condition and where patients with the disease typically present and receive treatment.

Ryan Haumschild, PharmD, MS, MBA: I’d like to turn to Dr Farberg for a minute to get your thoughts. What do you think makes generalized pustular psoriasis [GPP] a potentially life-threatening condition? And can you discuss maybe the varying levels of severity that present in your clinical practice?

Aaron Farberg, MD: First of all, I’ll tell you one of the many reasons I became a dermatologist is because I was always amazed at the complex interplay that the skin has I believe over 3000 diseases that dermatologists diagnose and treat. And that’s where you can really understand why and how the skin is perhaps the most important organ. Now, again, I may be biased as a dermatologist, but as Dr Lebwohl mentioned, GPP is actually a dermatologic emergency. These patients are often very sick and you’re dealing with a variety of issues such as sepsis. As we know, the skin is protecting us from bacteria and the outside environment and there’s an important interplay between the liver. You could have hepatic, respiratory, or renal failure. For these patients, it’s often necessary to have them admitted to the hospital. Also, as previously mentioned, you’ll have a variety of different electrolyte abnormalities; hypocalcemia or your inflammatory markers are up. There’s often an elevated ESR [erythrocyte sedimentation rate]. Your electrolytes could be imbalanced as well as your liver enzymes. Again, more reasons why these patients really require hospitalization and very close monitoring. Also, as mentioned, there is a variety of presentations. Thankfully, not all of our GPP patients arrive at the emergency room or the hospital, although a great majority of them unfortunately do. There are more mild cases and more localized cases. And for those types of patients, you will utilize different treatments. And sometimes the workup may take a little bit longer, but again, GPP is a dermatologic emergency and not just dermatologists need to know and understand this disease. It takes the emergency medicine doctor that’s seeing that patient initially, or the family practice doctor seeing and understanding this disease, so they know to call the dermatologist so that we can help these patients.

Ryan Haumschild, PharmD, MS, MBA: I think it’s a great way to differentiate that as much as it’s a dermatological situation that we need to address. At the same time, many practitioners might interact with patients. And I appreciate you two sharing the urgency of it. Again, life-threatening and rare disease, that’s not always educated to everybody. When you’re talking about something that really doesn’t have a huge incidence across the country, especially here in the United States, and practitioners that don’t see it all the time, how do they make that timely diagnosis and treatment to really help that course of disease progression and to minimize it? With that being said, Dr Lopes, I’d be curious as a leading medical practitioner on the payer side, you also get to see these rare diseases. How do you see patients with GPP typically presenting? And in what setting do you see treatment most likely occurring; inpatient, infusion, or at home?

Maria Lopes, MD, MS: It could be all of the above. I think as we just heard from two excellent specialists that treat these patients, these patients can present in the [emergency department (ED)], and they can present to the hospital. They’re often probably admitted from the [ED] if they have sepsis and septic shock. If they’re decompensated, they may be in the hospital for days, if not weeks, depending on how decompensated they are. And will they even require the ICU [intensive care unit]? I think they can also present to different specialists. There may be this diagnostic odyssey that may be happening and even misdiagnosis. I think this is a matter of education. I think education [is needed] for payers as well because this is really not on our radar whatsoever. This is a rare and very complex condition. When we hear psoriasis, we think of a very different patient subtype. And so, to hear life-threatening and why is it life-threatening is going to be important. The cost associated with this condition, depending on the spectrum and how often these patients flare, can be outpatient, it could be again, related to multiple specialists they’re seeing and laboratory evaluations. This can be a genetic condition. We’re now in the age of personalized medicine, so there could be diagnostic odyssey even related to genetic testing to look for this marker. But I think what will be important is how we not only educate but also confirm the diagnosis and then have a treatment approach that addresses the acuity of the life-threatening condition and, hopefully, have life-saving treatments that keep patients at home or if they’re presenting to an infusion center, the right setting so that hopefully we’re averting ER. We’re averting hospitalization and decompensation at that point.

Ryan Haumschild, PharmD, MS, MBA: You hit on a lot of great topics because I think one of them that I heard Dr Lebwohl talk about too is if we’re catching this in the hospital, how do we reduce that hospital stay? How do we get a therapeutic response in a timely manner? And how do we make sure we’re working with our payers to get approval to get access to that right drug? And I think those are the things that come together. And sometimes that’s something that we have to educate around a rare disease.

Transcript edited for clarity.

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