Generalized Pustular Psoriasis (GPP)

Generalized pustular psoriasis (GPP) is a rare, serious cutaneous disease that is challenging to treat; rates of morbidity and mortality are significant. Despite the wide range of accessible therapies, the overall quality of evidence-based research is limited by the lack of an algorithmic method for this disease.¹  

General Prognosis of GPP

As a whole, the clinical course of GPP is extremely varied, and when the disease is untreated, the course is prolonged and unstable. Although disease-free periods may last for years, they are usually interrupted by pustular flare-ups. Fortunately, advances in pathomechanics and novel targeted immunotherapy have vastly reduced the previously significant mortality rate, with more recent findings depicting a mortality rate of 4% to 7%.²

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Effects of Treatment on GPP Prognosis

A complete cure for GPP is not possible. Most patients must continue receiving treatment and avoiding disease triggers to avoid flares. Some patients respond to therapy, some have partial response, but many receive no benefit. Disease flare-ups can still be frequent. Early treatment is essential for preventing complications, such as congestive heart failure and infection. However, more severe cases of GPP are more prone to complications, and yet, they are harder to control with conventional therapies. Targeted immunotherapy may provide a new means of more effective response in these cases.² 

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Effects of Disease Flare-ups on GPP Prognosis

Patients with GPP frequently experience flare-ups, which are difficult to control and may become fatal. Because standard systemic therapies often have a slow onset of action, they may not adequately control acute disease manifestations. In addition, it is frequently difficult to resolve skin complaints completely. Oral retinoids are often recommended, but because of a number of side effects, including teratogenicity, elevated blood lipid and transaminase levels, mucocutaneous toxicity, hair loss, and skeletal abnormalities, their use is restricted.³ 

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Effects of Complications and Infections on GPP Prognosis

Cardiopulmonary failure in GPP may result in death, usually in untreated individuals. During the acute erythrodermic stage, sepsis and renal, hepatic, or cardiorespiratory failure can also cause death.⁴ To prevent potentially fatal consequences like sepsis, bacterial infection, and dehydration, an effective GPP treatment program must be implemented.¹ As long as significant secondary infections do not develop, the prognosis for children with episodes of GPP is favorable.⁴ 

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GPP Prognosis Related to Age

Older patients have a poorer prognosis than do younger patients.⁴ Aggressive treatment is required in older adults, especially those with von Zumbusch pustular psoriasis, to avoid serious complications. In addition, the risk of flare-ups is decreased with treatment.⁵ 

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GPP Prognosis Related to Disease History

The prognosis of patients with a history of chronic psoriasis vulgaris before GPP typically is better than the prognosis of those who have more atypical forms of psoriasis. Avoidance of precipitating factors and continued therapy is recommended for patients suffering from recurrent flares years after diagnosis.⁴

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References

1. Zhou LL, Georgakopoulos JR, Ighani A, Yeung J. Systemic monotherapy treatments for generalized pustular psoriasis: a systematic review. J Cutan Med Surg. 2018;22(6):591-601. doi:10.1177/1203475418773358
2. Boehner A, Navarini AA, Eyerich K. Generalized pustular psoriasis – a model disease for specific targeted immunotherapy, systematic review. Exp Dermatol. 2018;27(10):1067-1077. doi:10.1111/exd.13699
3. Reich K, Augustin M, Gerdes S, et al. Generalized pustular psoriasis: overview of the status quo and results of a panel discussion. J Dtsch Dermatol Ges. 2022;20(6):753-771. doi:10.1111/ddg.14764
4. Cockerell CJ. Pustular psoriasis. Medscape. Updated October 8, 2021. Accessed May 26, 2023. 
5. Pietrangelo A,Goldman L. What does pustular psoriasis look like?. Healthline. Updated September 30, 2019. Accessed May 26, 2023. 

Reviewed by Debjyoti Talukdar, MD, on 5/29/2023.

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Harshi Dhingra, MD

Harshi Dhingra is a licensed medical doctor with specialization in Pathology. She is currently employed as faculty in a medical school with a tertiary care hospital and research center in India. Dr. Dhingra has over a decade of experience in diagnostic, clinical, research, and teaching work, and has written several publications and citations in indexed peer reviewed journals. She holds medical degrees for MBBS and an MD in Pathology.