P107 Generalized pustular psoriasis: consequences of tedious procurement of spesolimab in Germany

Abstract Generalized pustular psoriasis (GPP) is a potentially life-threatening, autoinflammatory skin disease which is considered a rare and special variant of psoriasis. In limited disease, only local therapy may be applied, yet acute exacerbations and chronic remitting disease will demand systemic treatment. Various therapeutics like methotrexate, ciclosporin and corticosteroids as well as TNF-, IL23- and IL-17 blocking agents have been casuistically described or evaluated in small clinical studies, none of them being licenced so far by the FDA and EMA. Recently, the anti-IL-36 receptor antibody spesolimab was approved as a targeted treatment option for acute phases of GPP. However, the German Joint Federal Committee (Gemeinsamer Bundesausschuss) denied an additional benefit compared with systemic glucocorticoids, which is why the substance is not available in Germany anymore. Here, we report on a 47-year-old female patient who during the tedious application process for spesolimab had to be alternatively treated with long-term high-dose glucocorticoids resulting in a septic condition for several weeks and distinct Cushing’s disease. After final approval and drug delivery through an international pharmacy, she was successfully treated with spesolimab and could be tapered from systemic corticosteroids.