Generalized pustular psoriasis (GPP): a chronic inflammatory disease

Generalized pustular psoriasis (GPP) is a chronic inflammatory disease with a heterogeneous clinical course, including episodes of painful pus-filled blisters, and systemic symptoms, such as fever, fatigue and erythema (redness).¹

GPP is recognized as a separate clinical entity from other forms of psoriasis and the symptoms are unpredictable,²which greatly impacts the quality of life for people living with the disease, causing fear and anxiety.³

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

Watch the animation below to learn more about GPP including the symptoms, its prevalence, and the life-threatening nature of the disease. ³

Understanding GPP

The symptoms of GPP can present on a continuum, or as periods of flaring.⁴ During a GPP flare, individuals can experience rapidly disseminating painful skin manifestations. These appear over large areas of the skin, accompanied by redness, severe itchiness, and dry, cracked, or scaly skin.^5,6

Outline of person with physical symptoms of GPP, showing red patches covering the body with pus-filled blisters. Illustrations of other symptoms are present in the image, including a thermometer to indicate fever, a target to indicate pain, and a battery to indicate fatigue.

GPP symptoms are unpredictable. This often causes fear and anxiety and can have impacts on everyday life such as work and school, emotional health, social activities, and finances.

Potential risks and complications

Diagnosis of GPP can be difficult, and misdiagnosis is frequent. 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.⁷

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.¹Additionally, 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.⁸

Prevalence

Image of a globe with the following reference,1.76 to 124 patients per million people are affected by generalized pustular psoriasis.

Prevalence of GPP is low and varies considerably across geographical regions, ranging from 1.76 to 124 patients per million persons in reports from key countries, including France, Japan, Sweden and South Korea.³

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References:

1. Prinz JC, et al. J Eur Acad Dermatol Venereol. Prevalence, comorbidities and morality of generalized pustular psoriasis: A literature review. 2023;37(2):256–273.

2. Min Zheng, Denish Jullien, Kilian Eyerich. The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis. National Library of Medicine. 2022

3. Reisner DV, Johnsson FD, Kotowsky N, et al. Impact of generalized pustular psoriasis from the perspective of people living with the condition: Results of an online survey. Am J Clin Dermatol. 23:65-71, 2022.

4. Puig L, Choon SE, Gottlieb AB, et al. Generalized pustular psoriasis: A global Delphi consensus on clinical course, diagnosis, treatment goals and disease management. J Eur Acad Dermatol Venereol. 37:737-752, 2023.

5. Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 15:907-919, 2019.

6. Understanding Generalized Pustular Psoriasis Symptoms _ Boehringer Ingelheim.2024

7. Choon SE, Navarini AA, Pinter A. Clinical course and characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 23:21-29, 2022.

8. Hideki Fujita. Melinda Gooderham. Ricardo Romiti. Diagnosis of Generalized Pustular Psoriasis. 2022.