Generalized Pustular Psoriasis – The Unseen Burden and Need for Collaboration

21 February 2023

By Boehringer Ingelheim

Generalized pustular psoriasis (GPP) is a rare, unpredictable, and life-threatening autoinflammatory systemic disease that is distinct from plaque psoriasis. During a GPP flare, painful pus-filled blisters (called pustules) may appear suddenly over large areas of the skin, along with redness, severe itch, and dry, cracked, or scaly skin. People living with GPP may also experience more general symptoms, such as fever, headache, extreme tiredness, or a burning sensation on the skin. Due to this, there is a high unmet need within the GPP community and a desire to increase awareness of the significant burden of this debilitating disease.

Because of its rarity, misdiagnosis of GPP is common. Doctors and dermatologists hardly ever see a GPP patient, and patients may take several attempts to reach a correct diagnosis. The delay in diagnosis can leave patients feeling hopeless, especially if they aren’t given the correct information.

However, it’s not only misdiagnosis and delays that create a burden on patients:

“I was out with my son, and we went swimming... There were probably 8 - 10 children in the pool at the time. All of a sudden, a couple jumped out of the pool here, a couple jumped out there, and a mother came over and took her kids out. After, when it was all done, the only people in the pool were me and my son.” – Dale, living with GPP

The experience of Dale demonstrates the social stigma of living with a visible skin disease, which has a huge emotional impact. When Christine, who lives with GPP has a flare, she is prevented from doing many things she would normally do, like lifting her daughter up. This uncertainty can fill patients with anxiety, resulting in the mental burden of the disease often becoming as devastating as the physical impact.

“To have people that you love see you in that condition and have to help you do things like put your clothes on, or carry you somewhere, that is a difficult adjustment” – Christine, living with GPP

The theme of last year’s World Psoriasis Day was to highlight the unseen burden of living with psoriatic disease: mental health. Connecting communities across the world, we collaborated with IFPA to urge people to uncover the pain, stigma, and uncertainty behind psoriatic disease. We highlighted the impact of living with GPP through the eyes of people with the condition, focusing on how it impacts their emotional and mental health.

As part of this, we developed a fashion line based on the experiences of people living with GPP called The Unwearable Collection™. An internationally renowned textile designer, Bart Hess, collaborated with people living with GPP to create a four-piece collection that visualizes the experiences of a GPP flare. One of the pieces brings to life the physical and emotional isolation that GPP can cause.

“Increased GPP awareness that leads to accurate and faster diagnosis is one important step to improving the quality of life for people living with GPP” – Frida Dunger Johnsson, Executive Director, IFPA

In order to raise awareness and make a positive change, we must collaborate with others. This was exemplified amongst the GPP and rare disease community at the inaugural GPP Forum. The multistakeholder forum brought together a diverse range of experts from patients, and dermatologists to rare disease experts to collaborate on fresh ideas to instigate a positive change for people living with GPP. This resulted in a GPP Charter, a set of commitments that will deliver hope for the GPP community.

In 2023 and beyond, we will continue to collaborate with all stakeholders to unite together to help reduce the stigma of living with a rare skin disease, ultimately making a positive change to improve the quality of life for those living with GPP.