GPP Diagnosis and Treatment: What Every Dermatologist Should Know

In this episode of Topical Conversations, Dr Aaron Farberg and Dr Neal Bhatia explore the challenges and advancements in diagnosing and treating generalized pustular psoriasis (GPP). emphasizing the need to shift the paradigm in managing GPP from viewing it as an acute, episodic condition to recognizing it as a chronic disease that requires sustained treatment and management. 

The diagnostic challenge 

Dr Bhatia identified diagnosis as the foremost challenge in GPP management, noting that many patients present to primary care physicians, urgent care centers, or emergency departments where their condition is misdiagnosed as folliculitis, acute generalized exanthematous pustulosis, or drug eruptions. He explains that misdiagnoses when it comes to GPP are often the result of an awareness gap, as many health care providers are simply not trained to recognize it. Without a timely and accurate diagnosis, patients are frequently prescribed unnecessary antibiotics or steroids in the absence of dermatologic input. 

Dr Farberg highlighted the importance of collaboration between dermatologists and community physicians, urging primary care providers to involve dermatologists immediately when encountering pustules and rashes. He emphasized the need for dermatologists to make themselves accessible for same-day or next-day appointments to ensure patients receive the appropriate care without delays. 

Educating dermatologists on rare diseases 

Both stressed the importance of education on rare dermatologic conditions like GPP. Dr Bhatia noted that medical dermatology topics often receive insufficient attention at conferences due to their perceived rarity. However, he cautioned that dermatologists will likely encounter these conditions during their careers and must be prepared. He encouraged dermatologists not only to remain vigilant for GPP but also to educate their colleagues to bridge the existing knowledge gap. 

Advancements in GPP treatment 

Historically, GPP has been difficult to treat, but the FDA-approved therapy spesolimab has transformed the treatment landscape. Drs. Farberg and Bhatia discussed the availability of spesolimab in both intravenous (IV) and subcutaneous formulations, each serving a distinct role in managing the disease. 

The IV formulation is designed to treat acute flares, providing rapid control of the disease, while the subcutaneous version is intended for long-term maintenance, aiming to prevent flares altogether. Dr Bhatia noted that clinical trials for the subcutaneous formulation enrolled patients in remission to test its ability to maintain disease quiescence. He also highlighted the trial’s rescue component, where patients experiencing flares received IV spesolimab, often achieving prompt symptom relief. 

Dr Farberg underscored the importance of distinguishing between the 2 formulations, advising against substituting multiple subcutaneous injections for an IV dose during acute flares, as the absorption profiles differ. He reassured dermatologists that the IV option, included in the clinical study design, provides an effective fallback for flare management. 

Shifting the paradigm 

Both Drs Farberg and Bhatia emphasized the need to redefine how dermatologists approach GPP, noting that it’s not only an acute condition but a chronic disease that causes a significant quality of life decline for patients. He advocated for transitioning patients on other biologics to spesolimab, which directly targets the IL-36 pathway specific to GPP. 

Dr Bhatia highlighted the significant improvement in quality of life that spesolimab offers, allowing patients to move from crisis management to a state of controlled disease. 

Addressing access and coverage 

Access to spesolimab has been streamlined, with both Dr Farberg and Dr Bhatia commending the support systems in place to facilitate rapid availability of the drug. Dr Farberg noted that he has encountered no significant barriers to obtaining it for his patients, a promising step toward closing both the educational and access gaps in GPP care. 

A comprehensive approach 

With spesolimab, dermatologists now have a powerful tool to manage this complex and rare condition effectively. By combining timely diagnosis, interdisciplinary collaboration, education, and advanced therapies, dermatologists can redefine the standard of care for GPP, ensuring patients receive comprehensive treatment.