Addressing unmet needs in generalized pustular psoriasis: Real-world insights
07 Jan 2025
As a rare and heterogeneous autoinflammatory skin disease, generalized pustular psoriasis (GPP) currently lacks clear and consistent management guidelines, presenting treatment challenges particularly for patients experiencing recurrent flares. This article highlights global GPP data presented at the European Academy of Dermatology and Venereology (EADV) Congress 2024 and reports recent case series of Chinese and Italian patients with GPP flares treated with spesolimab, an interleukin (IL)-36 receptor antagonist.
Global challenges with GPP
The prevalence of GPP varies globally, ranging from 0.02 to 1.4 per 10,000 people across different countries. With a highly variable clinical course, GPP is characterized by chronic symptoms and periodic flares, during which rapidly spreading erythema is covered by sterile pustules, crust, and scales. Flares can also be associated with systemic symptoms and markers of inflammation. The severity of flares differs between patients and can vary from one episode to another. Without proper treatment, systemic inflammation associated with GPP may progress into life-threatening complications. [J Eur Acad Dermatol Venereol 2023;37:737-752]
Due to its rarity, GPP remains poorly understood with limited real-world data. In response to this knowledge gap, the retrospective, noninterventional chart review study, SCRIPTOR, was conducted in 2022–2023 to examine patient characteristics, disease course, and treatment patterns. [Warren BW, et al, EADV 2024, abstract 4671; Kokolakis G, et al, EADV 2024, abstract 4635]
Epidemiological insights from UK
According to UK’s SCRIPTOR data from 27 patients aged 18–86 years with a mean follow-up of 3.0 years, the most common risk factors for GPP were previous diagnosis of plaque psoriasis (65 percent), use of systemic corticosteroids or other medications (55 percent), and comorbidities (50 percent). In the SCRIPTOR cohort, the most common comorbidities were plaque psoriasis (58 percent), hypertension (46 percent), and psychiatric disorders (29 percent). [Warren BW, et al, EADV 2024, abstract 4671]
Treatment patterns indicated that topical corticosteroids were the most commonly prescribed at diagnosis (36 percent), while topical corticosteroids (23 percent) and retinoids (18 percent) were mostly commonly prescribed for flares. The main treatment option between flares was methotrexate (MTX; 31 percent), followed by retinoids (19 percent) and biologics (15 percent).
Notably, after diagnosis, 52 percent of patients experienced ≥1 flare, and 86 percent experienced multiple flares. The mean number of flare episodes was 2.4 and mean flare duration was 43.9 days. Infections and medication withdrawal were identified as the main triggers for flares. Hospital/emergency department (ED) admission was required in 85 percent of patients after GPP diagnosis.
GPP perspectives from Germany
German SCRIPTOR data from 33 patients aged 28–86 years with a 2.5-year mean follow-up showed similar trends. Plaque psoriasis history (73 percent) remained the primary risk factor. Almost all patients (97 percent) had ≥1 comorbidity, most commonly plaque psoriasis (66 percent) and hypertension (66 percent). [Kokolakis G, et al, EADV 2024, abstract 4635]
Echoing data from the UK, topical corticosteroids were the most frequently used treatment at diagnosis (34 percent) and for flare management (31 percent). However, unlike the UK cohort, biologics were the most commonly prescribed agents for long-term treatment of German patients (39 percent).
After diagnosis, 58 percent of patients experienced ≥1 flare, and 42 percent experienced multiple flares – rates that were lower than those in the UK cohort. Hospital/ED admission was required in 94 percent of patients after GPP diagnosis (flare-related, 36 percent).
Unmet needs
Study results from the UK and Germany revealed inconsistent treatment strategies for GPP flares and long-term management. The significant burden of GPP, characterized by both cutaneous and extracutaneous symptoms as well as recurrent flares, imposes a substantial strain on healthcare resources. Guidelines on GPP management, more effective treatment options, and education on optimal care are needed. [Warren BW, et al, EADV 2024, abstract 4671; Kokolakis G, et al, EADV 2024, abstract 4635]
