Bridging the gap in Generalized Pustular Psoriasis – a call for greater awareness and understanding

Imagine living with a rare, lifelong disease that is unpredictable and at any moment can flare up, needing emergency treatment. A disease that results in red, itchy, sore pustules that can occur all over the body.

Emmanuelle Clerisme-Beaty, former Head of Clinical Development and Medical Affairs, Dermatology, at Boehringer Ingelheim, reflects on insights from people living with generalized pustular psoriasis (GPP) and what this means for their care.

Imagine living with a lifelong disease that is unpredictable and at any moment can flare up, needing emergency treatment. A disease that can be triggered by sunlight, infections, stress or pregnancy. This is the reality for people living with Generalized Pustular Psoriasis (GPP).

GPP is a rare disease and its impact on those affected is immense. Patients experience widespread non-infectious pustules erupting on the skin, which are red, itchy, painful and of course highly visible to other people. And it goes much deeper than the skin – GPP flares can also cause fever, chills, fatigue and nausea, as well as considerable mental and emotional distress. Without prompt referral and treatment, GPP flares can lead to serious complications like sepsis and renal failure.

There is very little published information on the experience of those living with GPP. New data presented at the 2021 European Academy of Dermatology and Venereology (EADV) congress, highlights the experience of 66 U.S. patients and uncovers some of the gaps in care that are proving challenging to bridge.¹

Journey to diagnosis

Similar to many rare diseases, nearly 40% of the patients included in the study reported that it took years to receive an accurate diagnosis, and over half (51%) recalled visits to multiple healthcare professionals before this. One of the key reasons, cited by 59%, is initial misdiagnosis – GPP can often be mistaken for an infection, and this may lead to incorrect treatment or a delay in referral to a specialist.² Due to its rarity, many healthcare professionals do not have experience and knowledge of GPP, therefore improved recognition and understanding of GPP is essential to helping patients receive a prompt diagnosis and effective disease management.

Impact of GPP

GPP symptoms, triggers and flares can affect people in different and varied ways. The physical and mental health impact can affect all aspects of life, including daily activities, education, work and social relationships. The impact of flares cannot be under-estimated - most patients surveyed (86%) experienced two or more flares in the preceding year, and over three-quarters reported a medium-high impact on most aspects of daily living. Flares may last for weeks or months, and during this time, simply having a shower or going out can be difficult.

The majority of those questioned in the new research (71%) live in fear of experiencing a flare and two-thirds of patients are anxious about their disease. Emotional stress itself is reported to be a key trigger for a flare for 83% of those surveyed.¹

Bridging the gap in GPP

Despite GPP presenting differently between people, this latest study reveals how many patients share similar experiences in the journey to diagnosis, fear of flares, emotional stress and anxiety. These insights highlight the pressing need for faster diagnosis and treatments that can quickly alleviate symptoms. At Boehringer Ingelheim we are undertaking extensive research into this area and are committed to bringing treatments to patients where there is high unmet need.

It is important to help support people living with GPP to take an active role in understanding and managing their disease. Keeping a diary to track symptoms and possible triggers may help, and they should be encouraged to actively look after their mental health and wellbeing. Healthcare professionals can signpost to patient organization websites, such as the International Federation of Psoriasis Associations (IFPA), which can provide more information or connect patients with national organizations for local, practical or patient-to-patient support.

Click here to view our “Live well with GPP” infographic to understand how to support people living with GPP.

References

Nirali, K, et al. Insights from patients with generalized pustular psoriasis (GPP): Results of an online survey in the US 2021. Poster presented at European Academy of Dermatology and Venereology Conference. 2021.
Strober, B, et al. Unmet educational needs and clinical practice gaps in the management of generalized pustular psoriasis: Global perspectives from the front line. Abstract presented at World Psoriasis & Psoriatic Arthritis Conference. 2021.