Erin Boh, MD, shares her opinion on the importance of proactively stocking and streamlining generalized pustular psoriasis (GPP) medication pathways due to emergent care requirements of GPP.
Erin Boh, MD: I think that as we get more familiar with the drug, it’s going to hopefully be that way because there are people who do have chronic flares. And my patient, and I’m not sure about yours, and I’d love to hear about it. For mine, it was a medical emergency requiring inpatient care as we’re all taught in dermatology. This patient was in high-output failure, cardiac failure. She had multiple comorbidities, not something you can sit around at home and say, “Oh, well let’s try this, let’s try that.” Because she was literally crashing in front of us. And so, you really need to stick to it. And we did have a number of late calls into the midnight hour, I would say, for 2 days to try to get this facilitated. But it did work out. And when you see the photos, it makes you really happy because this patient is a 100% exfoliative erythrodermic with superimposed pustulosis. And it took 2 weeks, but 2 doses over the 2-week period. But she totally cleared, and she is still clear now, 7 to 8 months later now on stable psoriasis medicine.
Mark Lebwohl, MD: I will say that your illustration brings up a point, which I now have a lot of experience with, and that’s that colleagues around the country are calling. And then 1 insurance company says, “Well, you have to get a negative TB [tuberculosis] test.” For god’s sake. The patient is dying. In 2 days, the patient could be dead, but their TB test could be negative, or they ask for a skin biopsy. This is really a clinical diagnosis. You don’t need a skin biopsy to make that diagnosis. And to withhold life-saving treatment for a patient who literally could be dead in 2 days or 1 day, when you know the treatment’s there waiting to be given to them, is just a crime. So, when an insurance company asks you for a skin biopsy or a blood test, I believe that you’ll be seeing a position paper by a number of members of the board of directors of the AAD [American Association of Dermatology] and other interested psoriasis experts, which specifically says you don’t need a biopsy and just treat the patient when you see this life-endangering condition.
Erin Boh, MD: But what I teach my residents is, we did biopsy. And in fact, she was biopsied 4 times because there were such confounding factors; but the 1 word I use now all the time is pending. So, I said, “Oh, we have a biopsy, but it’s pending. And she’s got known psoriasis.” And I don’t worry about TB, but I think that’s another good explanation. Oh, it’s pending. But I do want to point out that in this case, at least for spesolimab, when you’re treating it, it blocks IL-36 [interleukin-36].
The cool thing about it is that there are no comorbidity restrictions. So, it doesn’t matter that the patient has significant renal disease. My patient’s creatinine went up to 4.6 and it was a 1.7 on admission. So, she was really cranking up the kidney failure. So, there are no contraindications in kidney disease, liver disease, even technically in infection. It doesn’t really work through that pathway. So, you don’t really need to worry as much about it. You want to exclude those as confounders and maybe drivers of pustulosis, but there are no contraindications to the drug, with the exception of if they had had hypersensitivity to the drug previously. In my opinion, and I don’t know if there are any contraindications that you can come up with. Obviously, we don’t want to give it when they’re actively infected, but it really should not make that much difference, I think.
Mark Lebwohl, MD: I agree with you completely. This is a life-threatening condition, and we have a life-saving drug that we should give despite other confounding factors. Now, I love your example of creatinine levels going up because it really tells you why patients who have generalized pustular psoriasis die. You lose all the protective functions of the skin. So, they come in, they’re often febrile or even hypothermic because you never think about your skin controlling your body temperature, but it has a big role in controlling your body temperature and you lose the integrity of the skin when you get generalized pustular psoriasis. Kidney function—well, your skin is a barrier against fluid loss. And…when you lose a lot of fluid, you can go into shock. Renal failure is not rare. It’s common. And to see the creatinine levels rise correlates with that loss of fluid.
So, this is one of those conditions where you have to keep strict iNOS [inducible nitric oxide synthase] and make sure that you’re maintaining the perfusion of the organs, including the kidneys. Many other things go wrong. All the patients who have generalized pustular psoriasis have low albumin because they lose protein through the skin. They almost always have microcytic anemia because they lose iron through the skin. They can develop electrolyte, abnormalities. Hypocalcemia is common, but the loss of other electrolytes is common too, which can lead to, arrhythmias, cardiac arrhythmias. And of course, I think you mentioned that she was in high-output cardiac failure.
Erin Boh, MD: She was.
Mark Lebwohl, MD: Your heart is working so hard to keep up with all the demands of this skin condition and the loss of fluids and everything else that it can’t keep up with it. Even though she may have a healthy heart, those patients end up with these grossly swollen legs. So often they’re in the ICU [intensive care unit], they’re shivering because their temperature control is off. They really look like they’re going to die in hours. And your patient took longer than most because the patients we’ve seen have turned around in hours. Literally the next day they look dramatically better. So, this is a life-saving drug, and we should try as much as possible to speed the delivery of it.
Transcript Edited for Clarity