Generalized pustular psoriasis (GPP): a rare, chronic, systemic disease¹

Increased awareness of the distinct generalized pustular psoriasis (GPP) symptoms may lead to better patient outcomes including more accurate diagnosis, improved treatment options, and reduced hospital stays. 

Generalized pustular psoriasis (GPP) flares 

During a GPP flare, individuals experience the sudden eruption of painful pustules. These pustules appear over large areas of the skin, accompanied by redness, severe itchiness, and dry, cracked, or scaly skin. People with GPP may also experience more general symptoms such as fever, headache, extreme tiredness, or a burning sensation on the skin.^1,3,4

Potential risks and complications

If left untreated, GPP can be life-threatening. Infections or inflammation of other organs may occur, leading to sepsis, cardiac, and renal failure. Any GPP flare should therefore be taken seriously as they can often lead to hospitalization.¹  

Causes 

The exact cause of GPP is not yet known, but it is believed to have a genetic component, with the IL-36 pathway playing a key role in the pathogenesis of the disease. Additionally, triggers such as stress, certain medications, or hormonal changes, e.g. during pregnancy, can contribute to the onset of GPP. It is important to note that GPP is not contagious and cannot be transmitted to others.¹ 

Prevalence 

GPP is a rare disease, and while epidemiological data vary between geographical region, ethnicity, and study methodologies, published estimates vary from 0.18–12.4 per 100,000 people worldwide.⁵ 

Recent estimates indicate a prevalence of approximately 9 per 100,000 in Sweden and the US, and around 3 per 100,000 in Japan.^6,7 

Diagnosis of GPP can be difficult, and misdiagnosis is frequent. As a rare condition, it is commonly confused with other psoriatic diseases, with a recent study identifying that 59% of people with confirmed GPP were initially misdiagnosed.⁸ 

Wider impact of a GPP diagnosis 

GPP not only affects the physical health of individuals but also has a significant impact on their daily activities, social interactions, and mental well-being. The years without a diagnosis, the intensity of a GPP flare, the constant worry of future flares, and people’s reactions to GPP symptoms can all add to the mental burden of the disease. Living with GPP may result in changes in socializing and interacting with loved ones, as well as feelings of anxiety and depression.⁸ 

The impact of GPP also goes beyond the individual, creating a wider effect on the family caring for someone with GPP and healthcare systems due to emergency care and extended hospitalization.⁹ 

To find out more about GPP visit: 

• GPPandME 

• The Unwearable Collection™ 

• About GPP Infographic 

References:

1. GPPandMe. Understanding Generalized Pustular Psoriasis. Available at: https://patient.boehringer-ingelheim.com/gpp/living-with-gpp (last accessed June 2023). 

2. Puig L, et al. J Eur Acad Dermatol Venereol. 2023. doi: 10.1111/jdv.18851.  

3. Crowley JJ, et al. Postgrad Med. 2021;133:330–44.  

4. Ly K, et al. Psoriasis (Auckl). 2019;9:37-42.  

5. Prinz JC, et al. J Eur Acad Dermatol Venereol. 2023;37:256–73. 

6. Löfvendahl, S., Norlin, J.M. and Schmitt-Egenolf, M. (2022), Prevalence and incidence of generalized pustular psoriasis in Sweden: a population-based register study. Br J Dermatol, 186: 970-976. https://doi.org/10.1111/bjd.20966.  

7. Feldman SR, et al. Prevalence of generalized pustular psoriasis in the USA: Results from multiple administrative claims databases. Presented at European Academy of Dermatology and Venereology (EADV) Congress 2021, virtual; 29 September–2 October 2021: Poster P0820.  

8. Reisner DV, et al. Am J Clin Dermatol. 2022;23(Suppl 1):65–71.  

9. Choon SE, et al. Clinical Course and Characteristics of Generalized Pustular Psoriasis. Am J Clin Dermatol. 2022; 23(1):21-29.