When treating generalized pustular psoriasis (GPP), it’s important to remember that “the goals…are to save the patient’s life and to reverse generalized pustular psoriasis as quickly and effectively as possible,” said Mark Lipwall, MD, dean of the College of Clinical Medicine. Treatments at the Icahn School of Medicine at Mount Sinai. He was the principal investigator for the spesolimab (Spevigo; Boehringer Ingelheim) trial published in The New England Journal of Medicine Which led to the US Food and Drug Administration (FDA) recent approval of the agent.
During Dermatology Times® A case-based partner’s perspective a seriesAnd Lebwohl discussed two cases of GPP and how he deals with the aggressive disease. First, see the case of a 28-year-old female nurse with a long history of psoriasis affecting only her elbows. She had a previous medical history of joint pain in her hands and a previous family history of psoriasis. After minimal success with nonsteroidal anti-inflammatory agents, her rheumatologist started her on 10 mg of prednisone. At this dose, my psoriasis cleared up completely, and it was reduced to 5mg, after which I suddenly started having flares.
After constantly fluctuating the prednisone dose, the patient began to develop erythema and blisters on more than 50% of her body. By the time Lebwohl treated her, she had taken more than 60 mg of prednisone for over 6 months.
“I still remember her shivering… one of the things that pustular psoriasis does… interferes with the normal functions of your skin. Most of us don’t even think about what our skin is doing until we’re introduced to a patient like this. She was hypothermic. Patients can have hypothermia or fever.” People with pustular psoriasis. [In addition,] She had swollen legs because she was suffering from heart failure. She is 28 years old. Her heart was normal, but it couldn’t keep up with the demands of this intense, intense inflammation. Her heart rate was 105 or greater,” Lebuhl noted.
His main conclusion from this patient is that systemic steroids should no longer be used to treat GPP. According to Lebwohl, between 1969 and 1971, two dermatologists named Ryan and Baker studied more than 100 patients in a trial that compared a folic acid analog — a drug similar to methotrexate — to systemic steroids for pustular psoriasis. Approximately a quarter of patients died in the systemic steroid arm.
“The most common cause of pustular psoriasis is withdrawal of systemic steroids. That’s why we generally try not to use systemic steroids in patients with psoriasis in general, but especially in patients with pustular psoriasis. That’s a lesson we learned from this case,” Lipole said.
One of the worst harmful effects of GPP which usually leads to death is the barrier that the skin creates against bacteria. It has been reported that many patients with GPP developed sepsis, specifically streptococcal sepsis. Lebohol explained that when treating patients with GPP, he monitors their heart closely because heart failure due to high blood pressure is common and difficult to treat, regardless of age or health status.
The next patient he discussed was a 64-year-old woman with a long history of plaque psoriasis. She had no known etiology, had a fever, blisters on 50% of her body, and swollen legs. I presented to a dermatologist with a fever of 102 degrees, body chills, painful edema of the lower extremities, heart rate of 105, and blood pressure of 90/60. When the patient was admitted to the hospital, she had a hematocrit 30 with MCV 70, serum albumin 3.5, calcium level 8, and serum creatinine 1.5.
At the time of admission, the patient was taking calcipotriene ointment twice a day and halobetasol ointment on weekends. She was also taking losartan for high blood pressure.
The important reason is [that] We have mentioned a number of medications that can cause psoriasis. In that list, we didn’t mention the ACE inhibitors which are known psoriasis triggers. It turns out that there are sporadic reports of angiotensin receptor blockers such as losartan, angiotensin receptor blockers, causing psoriasis, but they are few and far between.
Speaking about this patient’s treatment, Lebwohl mentioned spesolimab — a fast-acting drug recently approved for GPP — as the preferred option. What is unique is the mechanism of action [of spesolimab] Is that there is a group of patients who are born with a deficiency of the IL-36 receptor antagonist, a syndrome called DITRA. These patients have a clear mechanism explaining why IL-36 is involved in the course of psoriasis development. By blocking IL-36 or its receptor with drugs like spesolimab, this opens up an opportunity to save patients quickly. In fact, in the Phase 1 study, the majority of patients treated with a single injection of spesolimab were cleared quickly. By the eighth day, most of them were cleared.”
According to Lebwohl, spesolimab is currently the best treatment for GPP because it has proven positive results, has FDA approval, and providers have seen themselves that it works quickly. Patients with GPP can develop severe disease in a short time, so GPP screening is critical.
Lebwohl concluded the series by emphasizing that many of his colleagues are still unaware of the benefits of spesolimab, and that it is critical to spread the word to as many clinicians as possible. Every doctor should quickly learn about spesolimab and what it can be given with it, he advised, and be ready to get his or her paperwork ready to get spesolimab to patients as quickly as possible, because GPP is a life-threatening disease.
- Lebwohl M. Case-based expert perspectives on the management of generalized pustular psoriasis. The situation-based partner perspective. November 8, 2022. Accessed November 28, 2022. https://www.dermatologytimes.com/case-based-peer-perspective/case-based-expert-perspective-on-the-management-of-generalized-pustular-psoriasis