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Challenges in Diagnosing GPP


Drs Raj Chovatiya and Boni Elewski discuss a patient case and the challenges of diagnosing generalized pustular psoriasis (GPP), commenting on the many types of psoriasis and treatment.

Raj Chovatiya, MD, PhD: And so, I think that the dilemma here that, obviously, I was the treating physician…is the way we talked about moderate to severe generalized pustular psoriasis, it can be challenging in these cases where somebody isn’t overtly toxic and necessarily in the hospital in an emergency department setting. And I think that was the biggest challenge for us in terms of thinking about what do we call this based on the fact that this patient is not doing well, but they’re not sick-sick in the way we talked about with just the entire lake of pus over the entire body, but rather sort of areas that are coming and going definitely in a pustular fashion? What would your thought process [have] been, Boni, based on kind of everything that I’ve told you about this case?

Boni Elewski, MD: Well, you had a tough case. That’s very tough. And I think apremilast would have been a reasonable option if she had just palmoplantar pustulosis, but it sounds like she had much more than that. Did she have a history of plaque psoriasis too?

Raj Chovatiya, MD, PhD: Interestingly, she did not have a history of plaque psoriasis. So, nothing that she could recall that fit the description of what we had called plaque psoriasis.

Boni Elewski, MD: And the fact that spesolimab worked so fast tells you, you were on the right track. She had some dysregulation of the IL-36 pathway, or it wouldn’t have cleared her up. So, I think you nailed it, but that’s an example of a tough situation that you have to make a diagnosis and the key was you saw pustules, and when you saw the patient, she wasn’t as bad as when she was in the past. She was kind of waxing and waning.

Raj Chovatiya, MD, PhD: That’s exactly right. If anything, when I saw her, the pustules had burst and so I saw those raw, open, scaly areas without necessarily the overt pustules. And so, I was relying a little bit upon her cellphone photographs and what I had in front of me too. And so, this is the tricky part for when somebody has a more waxing and waning quiescent disease state, which I really do want to emphasize that this is very possible in somebody with a generalized form of pustular psoriasis. You may not touch them on the day but give you exactly what you want to really nail down that diagnosis.

Boni Elewski, MD: The disease apparently does, GPP [generalized pustular psoriasis] does historically wax and wane. I’ve taken care of patients who’ve had the disease from childhood, and it comes and goes, comes and goes, comes and goes. And the problem is that they can’t keep on cyclosporine. It’s just impossible to do. They’re going to end up with more problems than we can deal with. So, alternative treatments are really important. And I think what you showed is totally different than the patient I want to talk to you about, because mine was a no-brainer. I knew what to do. Well, you saw the pustules and you reacted because of pustules, but I don’t believe the IL-36 blocker works in just pure palmoplantar pustulosis. So, your patient had much more than that.

Raj Chovatiya, MD, PhD: I agree, and I think that part of the trickiness here was that she had involvement sort of in areas that were pretty close to the palms and pretty close to the soles, but there was involvement in the other parts, part of the arms and while it wasn’t happening in front of the trunk involvement too. And so, I think that was what provided the tipping point. …I remember the resident that was working on this case with me was that I think that when you kind of really thought about the totality of the history, it allowed us to go forward with treatment. And this is just a general point for us in dermatology all the time. We see cross-sections, so, people coming to see us at one point in time, and that tells us very little about the longitudinal course of disease. Unless you act, you may not see what you’re looking for, and therefore your conclusion may be totally different unless you actually get that information. So, this is true for GPP and with any other disease. The history is so important to understand what the trajectory of disease has been like. I can’t emphasize that enough. Even though we love the visual aspect of disease dermatology, that history sort of is necessary to make a lot of our diagnosis.

Transcript edited for clarity

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