EADV 2021: New Frontiers in GPP

Dr. Emmanuelle Clerisme-Beaty, former Associate Head of Medicine - Inflammation, Boehringer Ingelheim

It was great to catch up virtually with so many colleagues at EADV this year. These are challenging times, so it felt good to come together, even online, to focus our minds on addressing the unmet patient needs that remain in dermatology.

A particular focus for us this year has been Generalized Pustular Psoriasis (GPP), a rare, heterogenous and potentially life-threatening neutrophilic skin disease, with a significant burden for patients. There is limited awareness and understanding of GPP amongst the medical community and its estimated prevalence ranges from 0.27—4.6 per 10,000 people worldwide.¹

I believe there are two key issues directly affecting GPP patients: firstly, diagnosis is often delayed or GPP can be misdiagnosed, resulting in a frustrating and potentially harmful process for patients; secondly, there is no real consensus on treatment, with Japan being the only country worldwide to have national guidance on the management of GPP.

To explore these challenges and promote scientific discourse, we brought together the leading voices in GPP in a symposium entitled “Beyond pustules: exposing the burden of Generalized Pustular Psoriasis.” Bruce Strober (Clinical Professor of Dermatology at Yale University School of Medicine), who kindly chaired the session, differentiated GPP from plaque psoriasis, contrasting the IL-36 and IL-23/IL-17 pathways, and provided guidance to ensure timely diagnosis. Siew Eng Choon and Mahira Hamdy El Sayed walked us through some case studies demonstrating the extreme and systemic nature of GPP that impacts the quality of life for this patient population. In a review of the available treatment guidelines and data, Ulrich Mrowietz highlighted gaps in current treatment options and Luis Puig provided an overview of the current therapeutic development landscape in GPP, including the IL-36 pathway.

In the absence of consensus data, Strober and colleagues surveyed the Corrona registry dermatologists to assess their perspectives on the treatment of GPP and shared their findings at EADV.^2,3 Respondents estimated the average hospitalisation during a flare is 3-8 days² and patients often have residual disease post-flare. The research also revealed an interesting paradox in satisfaction with current treatments – whilst most respondents thought treatments were effective, many also reported that the time to respond was slow.³

My key take-aways from the symposium reinforced the need to investigate more effective approaches in the management of GPP. With our deep understanding of molecular pathways in inflammation, we are committed to pioneering scientific innovations that target, repair and prevent many rare diseases, such as GPP and palmoplantar pustulosis. To that end, we are excited to be collaborating with the IRASPEN registry,⁴ a prospective registry aimed to improve the understanding of pustular psoriasis, including GPP.

References

¹ Boehringer Ingelheim. GPP prevalence. Data on file. April 2019.

² Strober, B et al. Flare characteristics of generalized pustular psoriasis in North America: survey results from dermatologists in the Corrona Psoriasis Registry. EADV 2020, 28 October – 1 November 1, 2020

³ Strober, B et al. Perspectives on generalized pustular psoriasis treatment in North America: survey results from dermatologists in the Corrona Psoriasis Registry. EADV 2020, 28 October – 1 November 1, 2020

⁴ ClinicalTrials.Gov. International Rare And Severe Psoriasis Expert Network (IRASPEN). Available at: https://clinicaltrials.gov/ct2/show/NCT04359394?term=IRASPEN&draw=2&rank=1 Last Accessed: October 2020.