Clinical phenotypes of 504 patients with pustular psoriasis from Europe and Egypt: a multicentre observational study

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BACKGROUND: Pustular psoriasis (PP) comprises a group of rare autoinflammatory skin diseases characterized by sterile pustules. Previous studies have focused on cohorts in East Asia and the West, while North African populations have been underrepresented. OBJECTIVES: To describe the demographic and clinical features of a large cohort of European and Egyptian patients with PP. METHODS: We collected data on 504 patients with PP from 13 centres across Egypt and 5 European countries (Estonia, Germany, Spain, Switzerland and the UK); we then analysed their demographic profiles, clinical presentations and treatment histories. RESULTS: Of 504 patients with PP (424 Europeans and 80 Egyptians), 78 (15.5%) had generalized pustular psoriasis (GPP), 374 (74.2%) had palmoplantar pustulosis (PPP), 12 (2.4%) had acrodermatitis continua of Hallopeau (ACH) and 40 (7.9%) had other or overlapping PP subtypes. PPP was more prevalent among Europeans than among Egyptians (83% vs. 28%), whereas GPP was more prevalent among Egyptians than among Europeans (60% vs. 7%). Europeans included more women than men (80% vs. 40% with GPP, and 79% vs. 23% with PPP; both P < 0.001). The mean age (SD) of European patients was significantly higher than that of Egyptian patients [59.9 (16.8) vs. 36.1 (18.6) years with GPP and 62.2 (16.4) vs. 40.2 (13.8) years with PPP; both P < 0.001]. European patients with GPP had higher rates of psoriasis vulgaris (PV) than Egyptian patients with GPP (29% vs. 10%; P = 0.043). Treatment patterns varied, with ciclosporin being frequently used in Europe, while treatment with methotrexate and steroids was more common in Egypt. CONCLUSIONS: This study highlights significant variations between European and Egyptian patients with PP. European patients more commonly presented with PPP, whereas Egyptian patients had a higher incidence of GPP and fewer diagnoses of comorbid PV.

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