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The condition is rare, with limited epidemiological data and no standard treatment.
A new investigation form the University of Pennsylvania Perelman School of Medicine offered additional data on generalized pustular psoriasis (GPP).
Generalized pustular psoriasis is a rare, chronic orphan disease with limited epidemiological data and, to date, no standard treatment.
Investigators led by Joel M. Gelfand, MD, MSCE, Departments of Dermatology and Epidemiology, University of Pennsylvania Perelman School of Medicine, found that the condition was associated with continued health care utilization over time.
In their study, Gelfand and colleagues detailed the clinical charaxteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with generalized pustular psoriasis across the United States.
The retrospective, longitudinal case series focused on adult patients 18 years or older with a diagnosis of generalized pustular psoriasis that had been confirmed by a dermatologist.
Data on this patient population was gathered from January 1, 2007 to December 31, 2018. A total of 20 participating sites’ electronic health records were utilized, along with site-specific databases.
Only patients who met the European Rare and Severe Psoriasis Ex- pert Network (ERASPEN) consensus definition of generalized pustular psoriasis were eligible for the study.
As such, investigators enrolled a total of 95 adult patients, 67 (70.5%) of whom were women with a mean age of 50.3 years.
The index date (called the “initial encounter” in the study) was the first date of active disease within the reporting institution. Information on demographics, medical history, and disease course were collected for each patient.
Further, descriptive statistics were used to summarize baseline characteristics, past medical history, and information regarding clinical encounters and treatments, and duration of therapy was calculated for each patient and each systemic therapy for all patient-drug combinations with at least 1 subsequent visit after initiation of therapy.
Finally, logistic regression was used to evaluate the as- sociation of age and sex with hospitalization or emergency de- partment visit during follow-up.
During the initial encounter, investigators reported that 35 patients (36.8%) were hospitalized, 9 (9.5%) of whom presented with the condition at the emergency department. A total of 64 (67.4%) patients were treated with systemic therapies.
Among 57 patients who had no prior history of generalized pustular psoriasis, 29 (50%) were inpatient admissions or emergency department encounters.
Gelfand and colleagues added that most patients had pustules on the trunk and extremities. Pustules were also reported on the scalp, face, genitals, nail unit, and mucous membranes in a minority of patients.
Symptoms were common, with 59 patients (62.1%) reporting skin pain and 25 (26.2%) reporting joint pain. Additionally, tachycardia and fever were recorded in 16 (16.8%) and 9 (9.5%) of patients, respectively.
The most common comorbidities identified in the study were hypertension, depression, diabetes, chronic kidney disease, and hypothyroidism.
Regarding therapies, systemic antibiotics were used in 15 patients (15.8%), while systemic steroids were given to 19 (20%). Additionally, 23 patients (24.2%) received acitretin, 21 (21.1%) cyclosporine; 13 (13.7%), methotrexate; and 6 (6.3%) phototherapy. Of biologic treatments, 4 patients received infliximab, 4 adalimumab, and 3, ustekinumab.
Finally, 19 patients (35.8%) were hospitalized for generalized pustular psoriasis symptoms experienced a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6).
With their study, Gelfand and colleagues suggested that continued research was needed.
“Further prospective research is necessary to better understand treatment efficacy in patients with GPP,” the team wrote.
The study, “Evaluation of a Case Series of Patients With Generalized Pustular Psoriasis in the United States,” was published online in JAMA Dermatology