Generalized pustular psoriasis (GPP): Unmet needs, the reality of living with the condition, and future management

Understanding generalized pustular psoriasis

I was recently fortunate to interview Dr Bruce Strober, MD, PhD, Clinical Professor of Dermatology at Yale University School of Medicine and Central Connecticut, and editor-in-chief of the Journal of Psoriasis and Psoriatic Arthritis. We discussed generalized pustular psoriasis (GPP), a rare disease that is still relatively unknown by many in the medical community but one that can be life-threatening, and which has a profound impact on patients’ lives. 

Unseen challenges

EMMANUELLE CLERISME-BEATY: Thank you for taking the time to speak to me today. Can I ask you to describe some of the unseen challenges that are faced by people living with GPP? 

DR BRUCE STROBER: I think that access to appropriate healthcare, both during and between flares, is the number one challenge for patients. Many patients face a helplessness in being unable to access care from providers that are knowledgeable about GPP. Another issue, that goes beyond the physical pain and discomfort of GPP, is the fearful anticipation of, “when is the next flare?”. The unpredictability of the condition often leaves patients experiencing a lack of control. Even when they are in a relatively remitted state, patients are hampered by the thought of what might be next. 

CLERISME-BEATY: I agree, I think managing the unpredictable nature of the disease definitely poses a challenge for patients. I would say it even goes beyond how frequently, or when, the flares come, to a deep concern about how different the symptoms might be. This adds to the psychological burden that patients experience with the disease, which can sometimes be overlooked by physicians or family members. As you know, a lot of patients suffer from depression and anxiety; comorbidities that go beyond the skin. It's not just the visual impact of the disease, but their ability to function as part of society and feel confident that are also undermined. 

DR STROBER: Absolutely, and as you mention family members, I would really emphasize that it is not only patients that are affected by GPP. It can be a disabling condition where a person is incapacitated, often unable to work and even complete day-to-day tasks. So, beyond caring about the health of their loved one, GPP can also put additional burdens on family members.

"The helplessness you feel when you are suddenly seriously ill. That was difficult for me to endure, being completely disabled, going from a state of health to illness." – Elke, living with GPP

Awareness and understanding

CLERISME-BEATY: I wonder to what extent, if any, advances in GPP awareness and understanding have influenced the patient journey in recent years?

DR STROBER: There has been some influence on the patient journey, but I think there is more to be done here. A lot of dermatologists derive their knowledge of GPP and their therapeutic choices from what they know about chronic plaque psoriasis – a different condition. Most dermatologists won’t see many, or indeed any, GPP cases during their careers, which hampers their ability to treat patients. There is only so far ‘book-learning’ can take you; I think that treating patients brings a permanence to your knowledge base and allows you to build on it effectively. The absence of patient exposure leaves doctors lacking in awareness of the latest updates and treatment nuances relating to GPP. Rare diseases like GPP will always be plagued from a clinical treatment standpoint by the rarity of that disease. 

CLERISME-BEATY: Indeed, the low prevalence of GPP does mean that there is a lack of awareness in clinicians to diagnose it and refer patients to the right places. That is why I believe that more work is needed, because significant numbers of patients are still not being diagnosed early enough. We know that there can be several barriers to treatment, including lack of access to treatment, and lack of knowledge among treating physicians about treatment options. I hope that the expanding options for flare treatment have helped to raise awareness and put the importance of GPP diagnosis at the forefront for physicians.  

Future management

CLERISME-BEATY: So, following on from that question, what do you feel should be the focus for future GPP management? 

DR STROBER: Well, I have a very firm belief about this issue - there should not be flaring patients. There should be flares that are initially controlled and then the initiation of chronic management so that they have no more flares after an initial one. This means the patients would have little doubt that this day, or tomorrow, or next week is going to be good, because they have confidence in the therapy and their provider. We must move away from reactive approaches to GPP and medicine generally. I think that effective clinical care is preventative in patients who are at risk, and when we have tools like targeted therapies to manage flares and maintain remission, we absolutely need to use them.  

CLERISME-BEATY: That is a great aspiration!  This leads me into my next question, how would a comprehensive treatment meet these needs and reduce the disease burden on people living with GPP? 

DR STROBER: There isn't a great standard of care right now for GPP. Over the next five years we need to hone in on the ideal approach instead of what I would call a ‘Wild West’ approach, where every provider has a different, often not very evidence-based, system. If we can drive collaboration over time and administer effective treatment, we will arrive at a better, more evidence-based approach to patients with GPP that could then form the basis of a guideline.

You may read more of my conversation with Dr Strober, where we discuss the importance of support and collaboration in the GPP community, here. [hyperlink to Part 2]

Boehringer Ingelheim remain committed to helping people living with GPP to lead lives free of the impact of the condition.