OBJECTIVES:

Generalized pustular psoriasis (GPP) is a rare severe inflammatory skin disease characterized by recurrent episodes of fever, fatigue, episodic erythematous cutaneous eruptions with sterile cutaneous pustules formation on various parts of the body, and neutrophil leukocytosis. The objective of this study was to determine GPP prevalence and treatment patterns in Germany.

METHODS:

A three-round Delphi consensus panel with six German clinical experts was held between December 2021 and June 2022 using online questionnaires to describe the prevalence and management of patients with GPP. Consensus was defined as agreement of 75% of participants.

RESULTS:

5 of 6 experts were occupied in (university) hospitals and had >10 years of experience in treatment of GPP patients. Experts agreed that 0.75% of all psoriasis patients suffered from GPP. Prevalence was estimated at 0.12 per 10,000; reflecting to approx. 1,000 patients to Germany. Experts agreed that approx. 46% of GPP patients show a moderate disease course whereas 33% and 21% show a mild and severe course, respectively. Patients have 1.5 flares on average, with 60% of flares lasting less than 4 weeks. Approx. 29% of GPP patients receive biologics for first-line therapy while 32% are treated with systemic corticosteroids. TNF-α inhibitors are the most common used biologics (53% of all biologic 1^st line therapies). 70% of patients respond to first line therapy, while 30% do not. Most of the patients that do not respond to their 1st-line therapy change to 2nd-line therapy, while dosage adaption and additive therapy are less common. 56% of GPP patients are eligible for maintenance therapy.

CONCLUSIONS:

Results indicate that the estimated GPP population in Germany is within the range of previous published estimates. Biologics are often used as first line treatment with a relevant proportion of patients not responding to their therapy.