Generalized pustular psoriasis (GPP) is a rare, chronic, autoinflammatory skin disease characterized by painful widespread pustular eruptions. Patients with GPP experience a substantial physical, social, and emotional burden, with significant impacts on their quality of life. Historically, treatment for GPP in the United States mainly involved off-label use of therapies approved to treat plaque psoriasis. Dysregulation of the interleukin-36 (IL-36) pathway appears to play a key role in the pathophysiology of GPP. Spesolimab is a first-in-class humanized monoclonal antibody that binds specifically to the IL-36 receptor and antagonizes IL-36 signaling. In September 2022, spesolimab was approved by the U.S. Food and Drug Administration for the treatment of GPP flares in adults and was subsequently approved for GPP flare treatment in other countries across the world. Here, we describe 4 cases from our respective clinical practices, in which individuals with GPP were treated successfully with spesolimab. These cases demonstrate the difficulties experienced by patients with GPP regarding the disease burden and its effect on patients’ lives. These cases also illustrate some of the challenges that health care professionals may face when seeking to initiate spesolimab treatment, as well as highlighting the need for wider awareness of both the impact and severity of GPP and of the efficacy of spesolimab in GPP treatment.
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Anderson et al. (2024) studied this question.
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