Investigators Highlight Unmet Needs of Patients with Generalized Pustular Psoriasis

Carla Zema, PhD

Carla Zema, PhD, and a team of investigators conducted retrospective research to further the understanding of flares in patients with generalized pustular psoriasis (GPP) beyond single-center case studies which previously made up the extent of the topic's research.

The study revealed an unmet need among patients with this rare variation of psoriasis. Patients experience intermittent flares of generalized and localized noninfectious, neutrophil-containing pustules with associated erythema. The generalized flares are accompanied by systemic symptoms like fever, chills, malaise, anorexia, nausea, and severe pain.

This can lead to patients requiring emergency care, as these flares can last for months at a time and cause life-threatening complications. While mortality estimates vary, a recent review across 5 studies reported a range of 5%-32%. A multitude of studies have shown these patients face increased healthcare costs and a higher comorbidity burden compared with patients with plaque psoriasis and the general population.

A Comprehensive Analysis

Adult patients with generalized pustular psoriasis were included in this retrospective cohort sudy with the aim to assess GPP flares and treatment, as well as differences among patients with and without flares documented in the US electronic health records.

Investigators gathered data occurring in health records between July 2015-June 2020. Index GPP diagnosis was the initial occurence in electronic health records without a coded history of the disease for at least 6 months prior. They identified flare episodes by using an algorithm based on diagnosis coding, care setting, type of clinician, GPP disease terms, and flare terms and attributes.

Main outcomes and measures included flare episodes that were characterized by frequency of presentation in each patient and subsequently the care setting, type of managing specialist, associated symptoms, and the type of treatment implemented before, during and after the episode.

Investigators stratified patient data based on documented flares, and then analyzed comparisons between the groups, accounting for demographic charateristics, comorbidity burden, health care use, and treatments.

Evidence of Unmet GPP Treatment Needs

According to the resutls, 1535 patients with generalized pustular psoriasis were identified, with 66.3% women, and a mean age of 53.4 years. Flares were documented in 271 of patients, with a collective total of 513 flares. Those with flares had a 34% higher mean Charlson Comorbidity Index score compared to those without, and were almost 3 times more likely to have inpatient visits.

Additionally, patients who experienced flares were more than twice as likely to visit the emergency department (ED) and had an increased use of almost all treatment classes. The absence of dermatological treatment for 30 days before, during, or after a flare episode was observed in almost a quarter of episodes (24%).

Among flares identified in outpatient (53%), inpatient (36%), and emergency department (9%) settings, the most utilized treatments were topical corticosteroids (35%), opioids (21%), other oral treatments like methotrexate, cyclosporine, and tacrolimus (13%), and oral corticosteroids (11%).

"This cohort study suggests that there is significant unmet need for the treatment of GPP and its flares, as evidenced by patients seeking treatment in inpatient and ED settings, as well as the lack of advanced treatments," investigators concluded.

The study, "Understanding Flares in Patients With Generalized Pustular Psoriasis Documented in US Electronic Health Records" was published in JAMA Dermatology.