RESUMO
BACKGROUND: Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening skin condition characterized by flares comprising widespread sterile pustules and systemic inflammation. Both the rarity and heterogeneity of the disease have made GPP classification and standardization of clinical criteria challenging. Before the approval of spesolimab (IL-36R antibody) in 2022 there were no approved treatments in the USA or Europe for GPP flares. Treatment for GPP has amounted to off-label use of medicines approved to treat plaque psoriasis. Our aim was to describe the socio-demographics, clinical characteristics, and treatment patterns of patients with GPP in Spain. METHODS: Non-interventional, descriptive, multi-center, retrospective chart review of patients diagnosed with GPP in Spain. RESULTS: 56 patients (50% women) were included, with a mean (SD) age at diagnosis of 53.7 (20.5) and a mean (SD) time of follow-up of 3.7 (3.1) years. In 80% of patients, GPP diagnosis was associated with a flare and 67.3% had known risk factors for GPP [such as previous diagnosis or family history of plaque psoriasis, comorbidities, smoking or stress. Hypertension and plaque psoriasis were the most frequent comorbidities (44.6% each). The number of GPP flares per patient-year was 0.55 with (range 0-4) a mean (SD) body surface area involvement of 21.3% (19.1). The most frequent manifestations of GPP flares were pustules (88.5%), erythema (76.9%) and scaling (76.9%). Additionally, 65.4% of patients had plaque psoriasis, 53.8% had unspecified skin lesions, and 30.8% experienced pain. The treatments used for GPP flares were off-label conventional systemic drugs (75%), mostly corticosteroids, cyclosporine and acitretin. In the periods between flares, off-label biologics were used in 56.5% of patients. During the study period, 9 patients (16.1%) had at least one complication, and 5 of them required hospitalization. CONCLUSION: This is the first multi-center study in Spanish GPP patients. Most patients were in their fifties, with personal or family history of plaque psoriasis, stress, smoking and a wide range of comorbidities and complications. Even though the number of flares per patient/year was 0.55, there was variability between patients. Both off-label conventional systemics and off-label biologics were used for flare management without a clear treatment pattern.
