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Overview of Generalized Pustular Psoriasis


Mark G. Lebwohl, MD, gives an overview of generalized pustular psoriasis (GPP) and explains how it differs from the more common plaque psoriasis.

Ryan Haumschild, PharmD, MS, MBA: Welcome to this AJMC® [The American Journal of Managed Care®] program titled, “Generalized Pustular Psoriasis: Provider and Payer Considerations for Care.” I am Dr Ryan Haumschild, Director of Pharmacy Services at Emory Healthcare in the Winship Cancer Institute in Atlanta, Georgia. And joining me today in this discussion are my colleagues, Dr Mark G. Lebwohl, MD, Dean for Clinical Therapeutics and professor of Dermatology at the Icahn School of Medicine at Mount Sinai in New York, New York; Dr Maria Lopes, MD, MS, former Chief Medical Officer at MagellanRx in Cresskill, New Jersey, and Dr Aaron Farberg, MD, dermatologist and Mohs Surgeon at Bare Dermatology affiliated with Baylor Scott & White Health System in Dallas, Texas. Today, our panel of experts will provide an overview of generalized pustular psoriasis [GPP]; they will describe how the disease presents and manifests, discuss current standards of care for management, explore the patient perspectives of the disease, and discuss the evolving treatment landscape. Thank you and let’s begin.

First, let’s talk about generalized pustular psoriasis and the disease process, including the proposed causes, common presentations, and overall epidemiology. Dr Lebwohl, I’d like to start with you. What is generalized pustular psoriasis and how does it differ from common plaque psoriasis? And please talk a little bit about some of the signs and symptoms and how it is implicated, especially psoriasis vulgaris, in generalized pustular psoriasis.

Mark G. Lebwohl, MD: Generalized pustular psoriasis is a true dermatologic emergency and a life-threatening condition. Clinically, it is completely different than psoriasis. Psoriasis presents with sharply demarcated red scaling plaques; while deforming and debilitating, it doesn’t threaten your life immediately. Generalized pustular psoriasis presents with red skin that is covered in pustules that can affect the entire body, and it can compromise the skin functions that we rarely think about until we’re faced with a patient who has this condition. For example, a patient will come in; they may have a fever or they may be hypothermic because the skin protects the body against the loss of heat. The skin is also a primary barrier against external infection. Patients may develop staph [staphylococcus] sepsis. There have been many reports of death from staph sepsis in patients with pustular psoriasis. But [besides the worst outcome,] many other things happen to those patients. If you do routine blood work in patients, the CBC [complete blood count] will show that the patients are often anemic because they lose iron through the skin—a form of microcytic anemia. Their white blood count may be elevated, partly because of hemoconcentration. They lose fluid through the skin, so it’s artificially elevated, or it may be elevated because of sepsis. When you check the chemistry screen, what you see is the albumin is always low because patients lose protein through the skin. They may become hypocalcemic. They may have various electrolyte imbalances. Of course, the skin is a barrier against fluid loss, so patients may be hypovolemic, and they may go into shock. Often you’ll see a patient shivering due to the loss of temperature control, and their heart cannot keep up with the inflammatory demands of GPP. They will develop high-output cardiac failure. They’ll have swollen legs and they’ll look as if they’re going to die in a few days. And in fact, they are at risk of dying [soon]. GPP is different from plaque psoriasis, which is a chronic condition, lasting a long time. Note that GPP [also] can be mild and chronic. In most patients, it is mostly precipitated by something—for example, systemic steroids. In fact, the most common cause occurs when treating a patient with psoriasis who is prescribed systemic steroids for a variety of indications. When you try to lower the steroid dose, they break out into pustular psoriasis. [This is a] common presentation; it is easily distinguished from common plaque psoriasis.

Transcript edited for clarity.

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