Although generalized pustular psoriasis (GPP) is a rare disease, the chronic disorder lacks appropriate standard therapy and is associated with increased long-term health care resource utilization in the US, according to findings from a longitudinal case series analysis published in JAMA Dermatology.

According to the researchers of the analysis, there currently exists US epidemiological data on GPP from only a single report comprising 63 patients. To add to this knowledge, the investigators retrospectively evaluated a case series of 95 adult patients with GPP. The analysis was designed to evaluate clinical characteristics, the natural course of the disease, treatments, and healthcare utilization of this GPP population in the US.

Patients in the longitudinal case series were treated at 20 academic dermatology practices in the US from January 2007 to December 2018. In the cohort, nearly 71% of patients were women. The overall mean age of patients at presentation was 50.3 years. The mean follow-up period was 19.8 months.


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In all, 36.8% of patients were hospitalized during the initial encounter, while 67.4% received systemic therapies to manage their disorder. Systemic therapies included systemic antibiotics (15.8%), systemic steroids (20.0%), acitretin (24.2%), cyclosporine (21.1%), methotrexate (13.7%), and phototherapy (6.3%). In terms of biologic agents, 4 patients received infliximab, 4 patients received adalimumab, and 3 patients received ustekinumab.

During follow-up, approximately 35.8% of patients were hospitalized, which corresponded to a median rate of 0.5 hospitalizations per year. Additional GPP-specific emergency department (ED) encounters were reported in 8 patients. There were a median of 3.2 dermatology visits per year. Compared with men, women experienced a decreased risk for an emergency department visit or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83).

The researchers of the case series analysis noted that the retrospective design of the study may have led to certain study limitations, including missing information in the medical record. In addition, given that all patients were from academic medical centers, the generalizability of the findings may also be limited.

The investigators concluded that additional “prospective research is necessary to better understand treatment efficacy in patients with GPP.”

Disclosure: This clinical trial was supported by Boehringer Ingelheim. Multiple authors declared affiliations with the pharmaceutical industry. Please refer to the original article for a full list of disclosures.

Reference

Noe MH, Wan MT, Mostaghimi A, et al. Evaluation of a case series of patients with generalized pustular psoriasis in the United States. Published online December 8, 2021. JAMA Dermatol. doi:10.1001/jamadermatol.2021.4640