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European Psoriasis Week 2021: Spotlight on Generalized Pustular Psoriasis

 

Dr. Emmanuelle Clerisme-Beaty

This week is the first ever European Psoriasis Week, organized by EUROPSO. We want to take this opportunity to raise awareness about a rare neutrophilic skin disease, Generalized Pustular Psoriasis (GPP), which has a distinct clinical presentation and aetiology.

GPP places a significant burden on those living with the disease, yet it is still relatively unknown by many in the medical community.1,2,3

Our former Head of Clinical Development and Medical Affairs in Dermatology, Emmanuelle Clerisme-Beaty, answers a few frequently asked questions about this rare disease – read on to find out more about GPP, how to differentiate it from more common forms of psoriasis and the need for increased education to detect and diagnose this disease.

At Boehringer Ingelheim, our goal is to help people living with GPP who struggle with their disease every day and the physicians who are treating them. To find out more about GPP, please take a look at our infographic here.

What is generalized pustular psoriasis (GPP)?

GPP is a rare, heterogenous and potentially life-threatening neutrophilic skin disease. It is characterized by episodes of widespread eruptions of sterile, macroscopically visible pustules, that can occur with or without systemic inflammation.1,2 These episodes can lead to complications, such as heart failure, renal failure and sepsis, often requiring emergency care.1,2,3

GPP can be characterized either as a relapsing disease with recurrent flares, or a persistent disease with intermittent flares.4 The severity of symptoms during these flares can vary from patient to patient.1

Currently, the estimated prevalence in Europe is 2 per million people,1 with more females being affected than males.1,2,5


How is GPP different to plaque psoriasis?

While there may be some overlap in biological markers and clinical symptoms between GPP and the most common form of psoriasis, plaque psoriasis, also known as psoriasis vulgaris (PV)4,10, GPP is a distinct skin condition.4,13,17

While people living with GPP can also carry a diagnosis of PV,4 clinically, pustular eruptions are the hallmark of GPP,4 whereas PV primarily consists of well delineated plaques with grey/silvery white scaling, with rare pustular involvement.12

Pathologically, GPP is characterized by inflammation as a result of dysregulation in the innate immune system and is considered a neutrophilic skin disease.13,14 This is different from PV which is characterized by adaptive immune responses and is considered predominantly an autoimmune disease.16,17

Why is it important to differentiate GPP from plaque psoriasis?

Understanding and differentiating between these two diseases, can lead to tailored and effective management plans and treatment options that address the underlying needs of people suffering from GPP. This is especially relevant for the treatment of GPP flare. 

Prompt diagnosis of GPP may prevent morbidity from complications including sepsis, acute renal failure, congestive heart failure and acute respiratory distress syndrome.6,7,8

What causes GPP?

The exact cause of GPP is unknown, but several factors can trigger GPP flares. These include sudden withdrawal of steroids, exposure to certain medications, overexposure to sunlight, genetic mutations and dental or upper respiratory tract infections.1,5,18 GPP flares can also develop during pregnancy5,22 as well as during periods of stress and anxiety.1,5,15 But in many cases, it is not known what triggers the flare. Further research is needed on the cause of this disease.

How does GPP affect those diagnosed?

The key characteristics of GPP are episodes of widespread eruptions of pustules with inflamed, red skin, known as flares.4,5 This can develop suddenly and can progress quickly. GPP flares can be associated with a multitude of symptoms, including anorexia, chills, fever, malaise, nausea and pain.1 These episodes can develop into life-threatening complications such as heart failure, renal failure and sepsis.6,7,8

GPP can greatly affect a patient’s quality of life and can have a substantial impact on their ability to perform daily activities, including employment and social relationships. The sudden flares associated with the disease, as well as its appearance can affect the mental health of people living with GPP, which may lead to anxiety and depression.2,3,16 In addition, those with GPP may also experience other serious comorbidities.16

How can physicians identify GPP specifically?

At present, the diagnostic guidelines for GPP are limited and lack consistency in their diagnostic criteria. There is also no specific medical test to detect GPP, however, skin biopsies may help to differentiate diagnosis.18,19

As people with GPP often present themselves in emergency settings during a flare, physicians often rely purely on clinical presentation and evaluation of the patient medical history to identify and differentiate GPP.

The lack of the guidelines, testing and knowledge can make diagnosis very challenging, especially when time is of the essence with GPP.

What management options are available?

There are currently no approved GPP-specific therapies for GPP flares in Europe.20

There is a high unmet need for treatments that rapidly and completely resolve the symptoms associated with GPP flares and prevent reoccurrences, with an acceptable safety profile.2,21,22

What other information sources are there for physicians and people living with GPP?

The EUROPSO (The European Umbrella Organisation For Psoriasis Movements) and local psoriasis patient organization websites offer a wealth of information about GPP for physicians and people living with GPP.

References

  1. Benjegerdes KE, et al. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis (Auckl). 2016; 6:131–144.
  2. Kotowsky N, et al. Characteristics of patients with generalized pustular psoriasis compared with those with psoriasis vulgaris: A claims database study. 2020
  3. Pfohler C, et al. Psoriasis vulgaris and psoriasis pustulosa - epidemiology, quality of life, comorbidities and treatment. Curr Rheumatol Rev 2013;9:2–7.
  4. Navarini AA, Burden AD, et al. European consensus statement on phenotypes of pustular psoriasis. Journal of the European Academy of Dermatology and Venereology. 2017; 31:1792- 1799.
  5. Baker H, Ryan TJ. Generalized Pustular Psoriasis. British Journal of Dermatology. 1968; 80(12):771-793.
  6. Kelly-Sell M, Gudjonsson JE. (2016). ‘Overview of Psoriasis’, in Therapy of Severe Psoriasis. Elsevier, pp. 1-15.
  7. Practical Dermatology. Addressing Localized and Generalized Pustular Psoriasis—A Potential Medical Emergency. Available at: http://practicaldermatology.com/2010/11/addressing-localized-and-generalized-pustular-psoriasisa-potential-medical-emergency  [last accessed September 2019].
  8. Jeon C. et al. Generalized pustular psoriasis treated with apremilast in a patient with multiple medical comorbidities. Journal of the American Academy of Dermatology. 2017 Nov; 3(6): 495–497.
  9. Morita A. et al. Adalimumab treatment in Japanese patients with generalized pustular psoriasis: Results of an open-label phase 3 study. J Dermatol 2018;45:1371–1380
  10. Medline Plus. Generalized Pustular Psoriasis. Available at https://medlineplus.gov/genetics/condition/generalized-pustular-psoriasis/#frequency  Accessed 12 May 2021.
  11. Augey F, et al. Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. European Journal of Dermatology. 2006; 16(6):669-73.
  12. Jin H, et al. Clinical features and course of generalized pustular psoriasis in Korea. The Journal of Dermatology. 2015; 42(7):674-8
  13. Furue K, et al. Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis. Acta Derm Venereol 2018;98:5–
  14. Fujita H, et al. Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP. J Dermatol 2018;45:1235–1270.
  15. Kim WB, Jerome D, Yeung J. Diagnosis and management of psoriasis. Can Fam Physician. 2017;63(4):278-285.
  16. Gooderham MJ, et al. An update on generalized pustular psoriasis. Expert Rev Clin Immunol 2019;15:907–919.
  17. Johnston A, et al. IL-1 and IL-36 are dominant cytokines in generalized pustular psoriasis. J Allergy Clin Immunol 017;140:109–120.
  18. Ohkawara A, et al. Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background. Acta Dermato-Venereologica. 1996; 76(1):68-71
  19. Sampogna F, et al. Measuring quality of life of patients with different clinical types of psoriasis using the SF-36. Br J Dermatol 2006;154:844–849.
  20. Choon SE, et al. Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol 2014;53:676–684.
  21. Ly K, et al. Diagnosis and screening of patients with generalized pustular psoriasis. Psoriasis. 2019 Jun 20;9:37-42.
  22. Bachelez H. Pustular psoriasis and related pustular skin diseases. Br J Dermatol2018;178:614–618.
  23. Ratnarajah K, et al. Spesolimab: A Novel Treatment for Pustular Psoriasis. J Cutan Med Surg 2020;24:199–200.
  24. Golembesky, A. et al. Healthcare resource utilization (HCRU) in patients with generalized pustular psoriasis (GPP) in Japan: A claims database study. 2020 ISPOR Asia Pacific Seoul, South Korea.
  25. Kotowsky N, et al. Healthcare resource utilization (HCRU) in patients with generalized pustular psoriasis (GPP): A claims database study. 2020 ISPOR US Annual Meeting, Orlando, USA.

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