Case Based Expert Perspective on the Management of Generalized Pustular Psoriasis - Episode 3

Case 1: Treatment and Disease Management Goals

Goals of generalized pustular psoriasis treatment are reviewed by Dr Mark Lebwohl, where he comments on the need to consider patient disease severity and comorbidities.

Mark G. Lebwohl, MD: The goals of GPP treatment, are to save a patient's life and to reverse the generalized pustular psoriasis as quickly and effectively as we can. There's some things that happen when you are as sick as the patient I just showed you. When you are covered head to toe in erythema and pustules, you lose all of the protective functions of the skin that you never even think about. We sit here in skin that is doing a lot of things and we're not realizing that it is actively helping us in many ways. For example, patients will often lose fluid through the skin. They can go into shock, they can lower their blood pressure, they can cause kidney damage because of that loss of fluids. Before we had a treatment like spesolimab, we used to hospitalize patients with GPP. The point of that hospitalization was to keep track of their fluid balance to make sure they didn't go into shock. The skin prevents loss of iron through their skin so patients will often come in anemic and they have microcytic anemia because of loss of iron. They lose electrolytes through the skin, so patients can lose potassium through the skin, which might lead to cardiac arrhythmias. They lose protein through the skin. When you look at their blood parameters, the albumen is often low because of the loss of protein. They can lose calcium through the skin, which makes them hypocalcemic and associated with many of the adverse effects of hypocalcemia. The skin has a major role in temperature control, not losing heat through the body, and patients may come in either febrile or hypothermic because of that loss of heat.

Probably the worst effect of GPP and one that has led to the deaths of many of our patients that are reported historically is the barrier the skin creates against bacteria. Many patients with GPP are reported to develop bacterial sepsis, specifically staph sepsis. That's been reported many times. We reported a series of patients with erythrodermic psoriasis which has the same drawback that those patients develop staph sepsis. Here we are with the potential for staph sepsis. The patient can lose temperature control in common febrile, and then you have to wonder, does the patient have an infection or is this all from GPP which can present with a fever? There are real complicated factors in this patient. Because the disease can be deadly, the goal is to treat it as quickly as possible. Are these goals driven by the patient-provider or both? Obviously by both. The patient doesn't want to die and we don't want them to die. How do you determine goals of treatment when selecting a regimen that targets both joint pain as well as the skin? First of all, let me separate out the joint pain issue. We have an acutely, severely ill patient who we have to clear. We would think about their joints for long-term management but the key message here is clear the psoriasis of the skin as quickly as possible because that severe skin disease is dangerous. To what extent does a patient's chronic disease state and age impact treatment approaches? Quite a bit. First of all, one of the side effects I didn't mention is high-output cardiac failure. The heart is working so hard to maintain the circulation to the skin in this ill patient. These patients will often develop high cardiac failure. I've seen it several times. Patients develop pitting edema as if they were in heart failure, but their heart is normal. It's that it's overworking and it can't keep up with the demands of the generalized pustular psoriasis. In older patients, we are more concerned about their age, we're more concerned about their cardiac function. We're also concerned about their ability to manage something like an infection or renal failure triggered by the loss of fluids and so on. We take it seriously in every patient, but we're more concerned about elderly frail patients.

Transcript edited for clarity