For a study, it was determined that GPP is a rare, severe neutrophilic skin condition was characterized by a rapid widespread eruption of superficial sterile pustules with or without systemic inflammation. GPP flares, if left untreated, could be fatal owing to severe consequences such as cardiovascular failure and major infections. There was a lack of GPP-specific treatments authorized in the United States or Europe. The most often used non-biologic therapy for GPP included retinoids, cyclosporine, and methotrexate. Case reports and short, open-label, single-arm studies provided the majority of the data for the currently available therapy choices. Recent advances in the understanding of the pathogenic mechanisms of GPP, as well as the identification of gene mutations associated with the disease, paved the way for the development of specific targeted therapies that selectively suppressed the autoinflammatory and autoimmune mechanisms induced during GPP flares. Some biologic medicines that target critical cytokines implicated in the activation of inflammatory pathways, such as tumor necrosis factor-blockers and interleukin (IL)-17, IL-23, and IL-12 inhibitors, emerged as possible GPP therapies, with several already licensed in Japan. The evidence for the effectiveness of these medicines came mostly from small, uncontrolled studies.
The finding of IL36RN mutations and the essential involvement of IL-36 receptor ligands in the pathophysiology of GPP was a noteworthy recent achievement that highlighted critical treatment targets for the condition. Biologic drugs that target the IL-36 pathway had shown potential effectiveness in patients with GPP, ushering in a new era of GPP focused treatment.
Reference:link.springer.com/article/10.1007/s40257-021-00658-9
