ABSTRACT
INTRODUCTION: Generalized pustular psoriasis (GPP) is a rare, severe, immune-mediated and potentially life-threatening skin disease. The rarity, differential diagnoses, relapsing nature, skin and systemic symptoms, complications and limited therapeutic approaches for this disease pose a clinical and psychological burden on patients and their families. AREAS COVERED: Epidemiologic data of GPP in Chinese patients, including the disease prevalence and age of disease onset, as well as epidemiologic data in global populations were reviewed. Multiple proinflammatory cytokines are involved in the disease development and clinical presentation of GPP and the interleukin (IL)-36-mediated signaling pathway play a central role. Furthermore, loss-of-function mutations in IL-36 RN (encoding the IL-36 receptor antagonist) are associated with GPP, suggesting a potential drug target for developing a disease-specific therapeutic approach. Biologic agents, including IL-36 R targeted agents, are promising treatment options, especially as existing conventional therapies are inadequate. Chinese guidelines for the diagnosis and treatment of psoriasis recommend systemic and topical treatment options for GPP and disease complications, as well as for GPP during pregnancy and juvenile GPP. EXPERT OPINION: This review summarizes the epidemiology, pathogenesis, clinical characteristics, disease burden and management of patients with GPP in China, and also describes future treatment targets and related clinical trials.