GPP (generalised pustular psoriasis) is a chronic, systemic inflammatory condition characterised by high fever and general malaise. In GPP patients, there is diffuse erythema and swelling of the extremities, as well as numerous sterile pustules all over the body. GPP frequently relapses throughout one’s life and can be fatal. Recent findings of the underlying molecular genetic foundation of many cases of this illness have aided physicians and researchers in their knowledge of the pathomechanism of GPP. However, the therapeutic therapy of GPP continues to confront numerous obstacles and ambiguity, necessitating an evidence-based assessment of the current clinical data on the management of this diverse illness. The current study focuses on the problems of exact clinical diagnosis and clinical symptom assessment in GPP. Furthermore, we update and quickly review current molecular pathomechanisms underlying GPP as an autoinflammatory keratinization disease. Recent articles have shed light on the genetic origins of GPP patients, as well as ethnic variations in predisposing variables. Although the incidence of these pathogenic alleles varies by ethnicity, according to recent research, at most 60.5 percent, 5.9 percent, and 10.8 percent of GPP patients have mutations/variations in these genes. All of the reported biologics examined appeared to be efficacious and well tolerated.
Although evaluating treatment success from trials on a small number of cases is challenging, new data show that biologics can be a valuable, strong tool for reducing skin and systemic inflammation in GPP patients and enhancing their quality of life.
Reference:https://link.springer.com/article/10.1007/s40257-019-00492-0
