P099 Spesolimab use in effective treatment of generalized pustular psoriasis: a case report

Abstract The management of generalized pustular psoriasis (GPP) has been revolutionized by the development of the interleukin-36 receptor antagonist spesolimab. In the Effisayil clinical trial, spesolimab showed higher rates of pustule clearance at week 1 in comparison with placebo (Bachelez H, Choon SE, Marrakchi S et al. Trial of spesolimab for generalized pustular psoriasis. N Engl J Med 2021; 385: 2431–40). The Effisayil 2 study demonstrated that flare reduction was also superior for those treated with spesolimab vs. placebo (hazard ratio 0.16, 95% confidence interval 0.05–0.54; P < 0.001) over 48 weeks [Morita A, Strober B, Burden AD et al. Efficacy and safety of subcutaneous spesolimab for the prevention of generalised pustular psoriasis flares (Effisayil 2): an international, multicentre, randomised, placebo-controlled trial. Lancet 2023; 402: 1541–51]. Although now an approved therapy, it is currently undergoing National Institute for Health and Care Excellence (NICE) appraisal and can only be obtained through compassionate funding. Here we present the first patient, to our knowledge, with GPP to receive spesolimab within National Health Service (NHS) care. A 61-year-old British Asian man presented acutely unwell with a widespread inflammatory pustular eruption in 2015. He was managed as an inpatient with topical treatment and oral prednisolone. Histopathology demonstrated subcorneal inflammatory pustules. The initial diagnosis was thought to be acute generalized exanthematous pustulosis. He received oral methotrexate therapy but upon readmission with pustulation flare, diagnosis of GPP was confirmed and acitretin commenced. Over the next 8 years he had multiple inpatient stays (four), with one requiring high-dependency care. In June 2023, he presented with a widespread significant pustular flare, with comorbidities including cholecystitis and pancreatitis. Compassionate funding was secured for spesolimab. Initial administration of 900 mg intravenously was followed by rapid improvement, with no pustules noted at day 3. At day 12, he developed new pustules and a further 900 mg dose intravenous was administered. Following this, skin clearance without additional systemic treatment was maintained to 5 months. The patient then experienced widespread desquamative eruption and incipient pustulation, and as it was not possible to secure further compassionate funding for spesolimab as maintenance therapy, he commenced secukinumab therapy. He has subsequently maintained skin clearance, with Dermatology Life Quality Index 0, not requiring any further hospital admissions for skin flare. In our patient with GPP spesolimab was a highly effective treatment modality, leading to lasting skin clearance when used as monotherapy from its induction, but unavailable for maintenance therapy. We await NICE appraisal, due March 2025, to consider its use within NHS care.