Generalized pustular psoriasis (GPP) is a rare, severe, neutrophilic skin disease characterized by episodes of widespread eruption of sterile, visible pustules, which can occur with or without systemic inflammation and with or without plaque psoriasis. 1,2 GPP is associated with potentially life-threatening systemic complications including sepsis and cardiac failure, and is described by the Japan Ministry of Health as an intractable disease. [1][2][3][4] Mutations in IL36RN, CARD14, or AP1S3 have been found in patients with GPP. 5,6 Small Japanese studies have identified a number of different IL36RN mutations in patients with GPP. [7][8][9] Patients with these mutations may be at risk of earlier onset of disease and more severe disease. 10 Indeed, one small study (n = 31 patients with GPP)

Little is known about the disease burden, health‐care resource utilization (HCRU), or treatment of patients with generalized pustular psoriasis (GPP) in Japan. This retrospective cohort study used data from the Japanese Medical Data Center database to compare the demographics, comorbidities, and medication use of patients with GPP and plaque psoriasis and estimate their all‐cause HCRU. The patient selection period was from January 1, 2015 to December 31, 2019, and patients must have had at least one confirmed inpatient claim or outpatient claim for GPP or plaque psoriasis. During the 12‐month follow‐up period, 110 patients with GPP and 20,254 patients with plaque psoriasis were identified. An age‐ and sex‐matched (4:1) comparator control cohort, including members of the general population without a diagnosis of psoriasis (but allowing for a diagnosis of psoriatic arthritis), GPP, or palmoplantar pustulosis, was used. The most prevalent comorbidities in patients with GPP included allergic rhinoconjunctivitis, hypertension, and peptic ulcer disease. Patients with GPP were more likely to experience more comorbidities than those with plaque psoriasis, including asthma, chronic obstructive pulmonary disease, interstitial pneumonia, hyperuricemia and gout, tonsillitis, psoriatic arthritis, other psoriasis, and osteoporosis. Patients with GPP were more likely to be treated with a combination therapy than those with plaque psoriasis (65.5% vs 21.7%, respectively) and less likely to be treated with a topical medication alone (20.9% vs 50.8%). Patients with GPP had more outpatient visits than patients in the plaque psoriasis or matched control cohorts (mean [standard deviation], 14.8 [8.3] vs 11.0 [7.6] and 7.8 [7.2], respectively). They were also more likely to require inpatient hospitalization (24.5% vs 6.4% and 5.0%, respectively). Despite study limitations, patients with GPP in Japan were found to have a higher disease burden, including presence of comorbidities and medication use, than those with plaque psoriasis.

Background: Generalized pustular psoriasis (GPP) is a rare, severe neutrophilic skin disease with high unmet clinical need. The introduction of a GPP-specific International Classification of Diseases, 10th Revision (ICD-10), code has made it possible to generate a more accurate GPP patient profile. Objectives: To describe the characteristics and compare the patient profile and burden of disease of patients with GPP with patients with plaque psoriasis. Methods: A retrospective study was conducted using a US administrative claims database, the IBM® MarketScan® Research Database. The study took place between October 1, 2015, and September 30, 2018. Patients with at least 1 inpatient or 2 outpatient L40.1 (GPP) or L40.0 (psoriasis vulgaris) diagnostic codes were included for analysis. Outcome measures included descriptions of comorbidities, medication use, and healthcare resource utilization (HCRU) among GPP, plaque psoriasis, and general population (matched to those with GPP) cohorts. Results: Patients with GPP had more baseline comorbidities than those with plaque psoriasis and the matched cohort, including psoriatic arthritis (20.6% vs 6.4% and <0.1%) and hyperlipidemia (20.4% vs 16.3% and 11.8%). Patients with GPP also had greater medication use and higher HCRU than those with plaque psoriasis and the matched cohort. Conclusion: Patients with GPP generally experience more comorbidities, with higher HCRU, than patients with plaque psoriasis. Although the large dataset permitted identification of GPP patients with longitudinal follow-up, the lack of a validation algorithm for GPP is a limitation and a potential area for future research.

Background: Generalized pustular psoriasis (GPP) is a rare, severe neutrophilic skin disease with significant unmet clinical need. Historically, GPP has not been well characterized; however, the advent of the International Classification of Diseases, 10th revision, has made it possible to more accurately characterize patients with GPP. Objective: To describe the characteristics and estimate the burden of disease in patients with GPP compared with those with plaque psoriasis. Methods: A retrospective study was conducted using a US administrative claims database, Optum® Clinformatics® Data Mart, between October 1, 2015, and March 31, 2019. Patients with at least 1 inpatient or 2 outpatient diagnosis codes for GPP (L40.1) or psoriasis vulgaris (L40.0) were included. The main outcome measures included the percentage of individuals with comorbidities, medication use, and healthcare resource utilization (HCRU), which were compared between patients with GPP and those with plaque psoriasis and a general population comparator control cohort. Results: Overall, 1,669 patients with GPP were identified at baseline; most patients were female (67.6%). Patients with GPP had more comorbidities than those with plaque psoriasis, specifically metabolic disorders: hyperlipidemia and type 2 diabetes. Medication use for patients with GPP differed from those with plaque psoriasis—patients with GPP required more frequent use of antihypertensives and antibiotics than those with plaque psoriasis. Patients with GPP also had higher HCRU than those with plaque psoriasis. Conclusion: Although this study has limitations, it shows that patients with GPP have a high burden of illness that differs from patients with plaque psoriasis.

Incidence and prevalence of ON among patients with intermediate, posterior and pan uveitis were comparable and higher than the rates in patients with anterior uveitis.