Assessment of flare frequency and severity of generalized pustular psoriasis in Japanese patients: A retrospective chart review study

Background Generalized pustular psoriasis (GPP) is a rare and often refractory disease, GPP flares can be life threatening. The rarity of prevents the development evaluation effective GPP-specific treatments obstructs disease understanding. Objective To evaluate frequency severity clinical background treatment history patients with in Japan. Methods This retrospective, longitudinal, Japanese chart review study included diagnosed (according to Dermatological Association [JDA] diagnostic criteria), ?6 months continuous observation within 10 years protocol approval at sites. Primary outcomes: during follow-up. Secondary patient characteristics (at time initial diagnosis) Results Overall, 205 were included; 106/205 (51.7%) male, 146/205 (71.2%) aged <65 years. Few had family (7/155, 4.5%) or psoriasis-related diseases (6/120, 5.0%). At baseline, 36.1% (74/205), 30.7% (63/205) 33.2% (68/205) mild, moderate severe GPP, respectively; reported by 177/205 (86.3%), which mostly (52/205; 25.4%) (99/205; 48.3%). During follow-up, 70/205 (34.1%): 47/205 (22.9%) 1 flare 23/205 (11.2%) ?2. Among 106 events was 55 50 severe. median first 7.7 overall incidence follow-up 11.5/100 person-years. topical (195/205; 95.1%), systemic therapy other than corticosteroids (177/205; 86.3%) biologics (163/205; 79.5%) most frequently used. Conclusions over one-third experienced moderate-to-severe flares, despite available treatments. There remains an unmet need for options.