Clinical, Cosmetic and Investigational Dermatology

Dr Uwe Wollina, the former head of the department of dermatology and allergology at the Academic Teaching Hospital, Dresden welcome Drs. Merola and Amin to discuss the potential triggers of an acute GPP flare. GPP can be debilitating for patients, and they will discuss the different aspects of the burden of disease for patients with GPP.
Generalized pustular psoriasis (GPP) is the most severe form of pustular psoriasis and affects large areas of the body. GPP is a rare disease, and has a variable presentation; thus, its diagnosis is challenging. The onset of symptoms is rapid, with the appearance of painful skin erythema, followed by the widespread eruption of sterile pustules. Acute GPP (called a flare) is often accompanied by systemic symptoms, including high fever, pain in skin lesions, malaise, and fatigue. Approximately half of GPP flares require hospitalization, with an average inpatient duration of 10–14 days. GPP prevalence estimates range from approximately 2–124 cases per million persons, with a female predominance. The most common age of onset of GPP is 40–60 years, although cases have been described in younger adults and children. GPP affects every aspect of patients’ lives and has a high physical and psycho-social impact. Recent research on the interleukin-36 pathway associated with GPP led to the development of a GPP-specific treatment, spesolimab, which was approved by the US FDA in September 2022. This podcast explores the clinical presentation, disease course, and burden of disease in GPP, including differential diagnosis and common triggers of an acute flare.

Dr Uwe Wollina, the head of the department of dermatology and allergology at the Academic Teaching Hospital, Dresden welcome Drs. Clive Liu and Eingun James Song, who are both dermatologists in Washington State to discuss their experiences in treating generalized pustular psoriasis, also referred to as GPP.
Acute episodes of generalized pustular psoriasis (GPP), known as “flares,” are characterized by the widespread appearance of pustules with surrounding skin erythema, and are often accompanied by systemic symptoms. The clinical course of GPP is unpredictable, and symptoms vary in extent and severity; the disease may be relapsing-remitting with recurrent episodes of pustulosis, or be more persistent. The triggers that may lead to flares include withdrawal of corticosteroids, stress, pregnancy, and infections. GPP-specific assessment tools, such as the Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) and the General Pustular Psoriasis Area and Severity Index (GPPASI), were developed to evaluate the severity of disease, and to monitor the patient’s response to therapy during clinical trials. Spesolimab is the first GPP-specific treatment available in the United States for the treatment of GPP flares in adults, and was approved by the US FDA in September 2022. To date, spesolimab has been approved by regulatory agencies in almost 40 countries, including Japan, Mainland China, and the European Union. Spesolimab is a first-in-class humanized monoclonal antibody that targets the interleukin-36 receptor, and blocks the downstream effects of the interleukin-36 pathway, which is associated with GPP pathogenesis. Data from clinical trials demonstrate the safety and efficacy of spesolimab in providing rapid clinical improvement for patients with GPP flares. Standardized international guidelines for the diagnosis and management of GPP are needed, and no recent GPP guidelines are available in the US. This podcast discusses clinical assessment tools for GPP (GPPGA and GPPASI), the evolution of GPP management guidelines, the therapeutic landscape of GPP, efficacy and safety data for spesolimab, and examines important considerations for patients living with this condition.

Dr Uwe Wollina, the head of the department of dermatology and allergology at the Academic Teaching Hospital, Dresden welcome Drs. Bhutani and Hawkes, both board-certified dermatologists in California to discuss their experiences in treating generalized pustular psoriasis, also referred to as GPP.
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 with GPP. 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 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.