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Generalized Pustular Psoriasis (GPP)

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Life Expectancy

Generalized pustular psoriasis (GPP) is a severe and rare form of psoriasis characterized by widespread pustules on the skin, often accompanied by systemic symptoms.1,2 

Although generalized pustular psoriasis can significantly affect a patient’s quality of life, specific data on life expectancy are limited. Studies examining mortality in patients with psoriasis suggest that the disease’s severity and associated comorbidities can influence mortality risk.3

Mortality Rates

Generalized pustular psoriasis is a chronic and potentially life-threatening condition, especially when associated with systemic inflammation and complications. Life-threatening complications that patients with GPP can develop include renal, hepatic, respiratory, and cardiac failure, along with sepsis. The systemic inflammation in GPP may lead to these complications, which can further affect overall health and mortality.2,4

Patients with GPP may have multiple flares in a year or experience a flare every few years. Most of these flares last for 2 to 5 weeks, and about 50% of them require hospitalization. Patients may remain in the hospital for 10 to 14 days.2

The mortality rate linked to GPP or its treatment, particularly the use of systemic corticosteroids, ranges from 2% to 16%.2 Reported numbers vary considerably, from 0 to 3.3 deaths per 100 patient-years.4 Recent data derived from Japan showed an overall in-hospital mortality rate of 4.2% among 1513 patients with GPP, and the mortality rate was higher in patients who received systemic corticosteroid monotherapy. Patients treated with biologics presented the lowest mortality rate.2GPP in pregnancy can be a life-threatening condition both for the mother and for the fetus.5

Read more about GPP treatment

Prognosis in GPP is better when a clear trigger can be identified. When there is no effective treatment, death can occur in the acute stage. The prognosis is better in children and for the subacute annular and circinate generalized pustular psoriasis types. GPP that develops from acrodermatitis continua of Hallopeau has the worst prognosis.1

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Impact of Comorbidities on Life Expectancy

Generalized pustular psoriasis is associated with multiple comorbidities, which can affect life expectancy, including hyperlipidemia, hypertension, diabetes mellitus, obesity, anxiety, and depression.2 

The comorbidity burden has been shown to be increased in patients with GPP when compared to the general population and patients with psoriasis vulgaris. In this study, comorbidities in GPP included hypertension, psoriatic arthritis, type 2 diabetes, and hyperlipidemia. Other conditions such as allergic contact dermatitis, Crohn’s disease, and obesity were also reported to be more common among patients with GPP.6

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Preventive Measures Against Mortality in GPP

Early diagnosis, appropriate treatment, and comprehensive management can help improve outcomes in individuals with GPP. Regular monitoring of associated comorbidities and implementing lifestyle modifications, such as maintaining a healthy weight, exercising, and managing stress, can help reduce the risk of complications and improve overall health.7-9 

It is also essential for patients with GPP to work closely with their dermatologists and other health care providers to manage their condition. A care team providing close guidance can promptly and effectively address symptoms and comorbidities that occur during the course of the disease.10

Read more about GPP care team

References

  1. Mirza HA, Badri T, Kwan E. Generalized pustular psoriasis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2023. Updated September 12, 2022. Accessed May 30, 2023.
  2. Choon SE, Navarini AA, Pinter A. Clinical course and characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):21-29. doi:10.1007/s40257-021-00654-z
  3. Papadavid E, Katsimbri P, Kapniari I, et al. Prevalence of psoriatic arthritis and its correlates among patients with psoriasis in Greece: results from a large retrospective study. J Eur Acad Dermatol Venereol. 2016;30(10):1749-1752. doi:10.1111/jdv.13700
  4. Prinz JC, Choon SE, Griffiths CEM, et al. Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review. J Eur Acad Dermatol Venereol. 2023;37(2):256-273. doi:10.1111/jdv.18720
  5. Trivedi MK, Vaughn AR, Murase JE. Pustular psoriasis of pregnancy: current perspectives. Int J Womens Health. 2018;10:109-115. doi:10.2147/IJWH.S125784
  6. Löfvendahl S, Norlin JM, Schmitt-Egenolf M. Comorbidities in patients with generalized pustular psoriasis: a nationwide population-based register study. J Am Acad Dermatol. 2023;88(3):736-738. doi:10.1016/j.jaad.2022.09.049
  7. Strober B, Leman J, Mockenhaupt M, et al. Unmet educational needs and clinical practice gaps in the management of generalized pustular psoriasis: global perspectives from the front line. Dermatol Ther (Heidelb). 2021;12(2):381-393. doi:10.1007/s13555-021-00661-2
  8. Live well with generalized pustular psoriasis (GPP): a collection of tips and techniques to help manage your GPP. Boehringer Ingelheim. Accessed May 30, 2023. 
  9. Krueger J, Puig L, Thaçi D. Treatment options and goals for patients with generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):51-64. doi:10.1007/s40257-021-00658-9
  10. Generalized pustular psoriasis: getting a diagnosis. Genetic and Rare Diseases Information Center (GARD). Updated February 2023. Accessed May 30, 2023.

Reviewed by Kyle Habet, MD, on 5/30/2023.

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