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Generalized Pustular Psoriasis Is Rare, Has Variable Treatment

More than 20 therapies were tried in a case series of 95 patients; 19 percent reported hospitalization during follow-up

By Dermsquared Editorial Team | December 08, 2021


Generalized pustular psoriasis (GPP) is a rare disease and has no standard treatment, according to a brief report published online Dec. 8 in JAMA Dermatology.

Megan H. Noe, M.D., M.P.H., from Harvard Medical School in Boston, and colleagues describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP in a retrospective case series involving 95 patients who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP. Patients were treated between Jan. 1, 2007, and Dec. 31, 2018.

The researchers found that 67 of the patients were women (70.5 percent). Overall, 36.8 percent of patients were hospitalized in the initial encounter and 67.4 percent received systemic therapies. More than 20 different systemic therapies were tried overall. Nineteen of the patients (35.8 percent) reported hospitalizations during the follow-up period, with a median rate of 0.5 hospitalizations per year. The risk for emergency department or hospital encounter was reduced for women (odds ratio, 0.19).

"These results further support prior findings that GPP is a rare disease and treatment is highly variable," the authors write. "There is no consensus for the most effective therapy for GPP, and as a result, more than 20 treatments with relatively short treatment durations were reported across patients."

Several authors disclosed financial ties to the biopharmaceutical industry.

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