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Jason E Hawkes,1,* Dale V Reisner,2 Tina Bhutani3,* 1Department of Dermatology, University of California at Davis, Sacramento, CA, USA; 2Patient Author, Yankton, SD, USA; 3Psoriasis and Skin Treatment Center, Department of Dermatology, University of California at San Francisco, San Francisco, CA, USA*These authors contributed equally to this workCorrespondence: Tina Bhutani, Email tina.bhutani@ucsf.eduAbstract: Generalized pustular psoriasis (GPP) is a rare, chronic, and debilitating disease characterized by flares of widespread erythema, desquamation, and pustule formation. GPP flares can be accompanied by systemic symptoms including fever, fatigue, malaise, and skin pain; severe cases may be fatal if untreated. Although GPP may occur concurrently with plaque psoriasis, they represent two distinct inflammatory conditions. Patients with GPP experience a substantial burden of disease, and the impact of GPP on an individual’s mental health and quality-of-life (QoL) goes far beyond skin pain and discomfort. The rarity of GPP may result in a misdiagnosis, as the sudden onset of skin pustules may be mistaken for a primary infection. Misdiagnosis with a subsequent delay in treatment has tremendous negative consequences for the affected patient. In September 2022, spesolimab became the first FDA-approved medication in the US for the treatment of GPP flares in adults. Spesolimab has since been approved by regulatory agencies in numerous countries, including Japan, Mainland China, and the EU. Prior to spesolimab, the clinical management of GPP relied on the off-label use of systemic or biologic therapies approved for plaque psoriasis or other inflammatory conditions. There is a need for increased education among healthcare providers regarding the clinical diagnosis, risk stratification, and therapeutic management of this rare disease, including the other novel GPP-specific therapies in development. In this podcast, two dermatologists and a patient w

Tina Bhutani,1,* Jason E Hawkes2,* 1Psoriasis and Skin Treatment Center, Department of Dermatology, University of California at San Francisco, San Francisco, CA, USA; 2Department of Dermatology, University of California at Davis, Sacramento, CA, USA*Both authors contributed equally to this paperCorrespondence: Dr. Jason E Hawkes, Email hawkes3@gmail.comAbstract: Generalized pustular psoriasis is a rare presentation of psoriatic disease and is characterized by the acute onset of diffuse superficial pustules on the skin. These pustules can often coalesce, forming what’s known as ‘lakes of pus’ that are most often seen on the trunk and on skin folds. GPP flares are often accompanied by systemic symptoms, including fever, malaise, and edema. The interleukin (IL)-36 pathway plays a central role in the development of GPP, although several other genes may be associated. The rarity of GPP makes its diagnosis challenging and it could be mistaken for an infectious condition or other types of pustular psoriasis, including unstable forms of plaque psoriasis that may present with pustules. Performing a thorough skin examination and obtaining a detailed history are vital to exclude these differential diagnoses. Incorrect or late diagnosis, inadequate or delayed treatment, and lack of specialist referrals may contribute to increased disease severity and can have a debilitating impact on patients’ quality of life. In this podcast, two US-based dermatologists discuss the clinical characteristics of GPP, highlight the central role of IL-36 in immunopathogenesis, and share practical approaches to recognizing and diagnosing the disease.Keywords: Diagnosis, generalized pustular psoriasis, genetics, immunopathogenesis, interleukin-36 pathway, quality of life