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Generalized Pustular Psoriasis (GPP)

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Comorbidities

Generalized pustular psoriasis (GPP) is a rare inflammatory skin condition characterized by sudden, recurrent, and widespread eruptions of multiple sterile pustules. These severe and potentially life-threatening flares can occur with or without plaque psoriasis or systemic inflammation (fever, malaise, and leukocytosis).1

Studies on Comorbidities of GPP

2022 Swedish Study

A nationwide study using data from the Swedish National Patient Register investigated the comorbidity profiles of patients with GPP from 2004 to 2015 and compared them with the profiles of the general population and patients with psoriasis vulgaris (PV). The study results showed that the comorbidity burden was higher in patients with GPP than in the general population and patients with PV.2 

The most prevalent conditions found in individuals with GPP included hypertension, psoriatic arthritis, type 2 diabetes, and hyperlipidemia. Allergic contact dermatitis, Crohn disease, nonalcoholic fatty liver disease, nephritic nonhypertensive disease, and obesity were more likely to occur in persons with GPP than in the general population.2 

In comparison with patients who had PV, those with GPP were more likely to have nephritic nonhypertensive disease, Crohn disease, chronic renal failure, diabetes type 1 or 2, peptic ulcer disease, and psoriatic arthritis.2

Read more about GPP clinical features

2021 Japanese Study

Another nationwide study, which used data from the Japanese Medical Data Vision database between 2015 and 2019, found that comorbidities were more likely in patients with GPP than in those with plaque psoriasis. The comorbidities included psoriatic arthritis, other forms of psoriasis, peptic ulcer disease, osteoporosis, interstitial pneumonia, hypertension, type 2 diabetes, hyperuricemia (gout), chronic obstructive pulmonary disease (COPD), obesity, asthma, insomnia, and thyroid disorders.3

2022 Spanish Study

A multicenter Spanish study of 24 patients with GPP found associated comorbidities such as dyslipidemia (58.3%), hypertension (45.8%), and psoriatic arthritis (41.7%).4  

Read more about GPP treatment

2013 Malaysian Study

A study by Choon et al. analyzed 102 Malaysian patients with adult-onset GPP diagnosed between 1989 and 2011. The researchers found a high prevalence of comorbidities in patients with adult-onset GPP, including obesity (42.9%), hypertension (25.7%), hyperlipidemia (25.7%), diabetes mellitus (23.7%), ischemic heart disease (8.8%), and hepatitis B (3.9%).5

Read more about GPP diagnosis

2012 Single-Center Study

In a single-center, retrospective study by Borges-Costa et al., approximately 47% of the patients had liver enzyme abnormalities, and a positive correlation was noted between total bilirubin and absolute neutrophil count. In a comparison of patients with acute GPP who had liver enzyme abnormalities and those who did not, a higher leukocyte count and a male preponderance were found in those with liver abnormalities.6

Studies have also found hypoparathyroidism to be associated with GPP.7

Arthralgia/Arthritis 

Several studies have reported arthralgia (joint pain and stiffness), arthritis (inflammation of joints), and psoriatic arthritis in patients with GPP. In Japanese studies, arthralgia or arthritis was the most common comorbidity in patients with GPP (reported in 8.3% to 50% of patients).8-10

Read more about GPP signs and symptoms

Geographic Tongue

GPP is often associated with lesions of the oral mucous membrane lesions, particularly on the tongue, a condition termed geographic tongue. In a study investigating geographic tongue, the prevalence was greater in both a GPP patient cohort (83.9% cases) and a GPP family member cohort (47.8% cases) than in a control cohort.11

In addition, geographic tongue has been reported to be more prevalent in patients with GPP in Asian studies than in patients with GPP in German and American studies.12

Read more about GPP clinical trials

Psychological Comorbidities

In a recent online survey that aimed to assess the effect of GPP on daily activities, completed by 66 American adults with GPP, the prevalence of psychological comorbidities such as depression (reported in 42.4% of respondents) and generalized anxiety disorder (reported in 36.4% of respondents) was increased.13

In another recent study of North American adults with GPP or plaque psoriasis, anxiety and depression were more prevalent in patients with GPP (reported in 38.3% cases) than in those with plaque psoriasis (25.8% of cases).14

References

  1. Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 2019;15(9):907-919. doi:10.1080/1744666X.2019.1648209
  2. 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
  3. Morita A, Kotowsky N, Gao R, Shimizu R, Okubo Y. Patient characteristics and burden of disease in Japanese patients with generalized pustular psoriasis: results from the Medical Data Vision claims database. J Dermatol. 2021;48(10):1463-1473. doi:10.1111/1346-8138.16022
  4. Rivera-Díaz R, Daudén E, Carrascosa JM, Cueva P, Puig L. Generalized pustular psoriasis: a review on clinical characteristics, diagnosis, and treatment. Dermatol Ther (Heidelb). 2023;13(3):673-688. doi:10.1007/s13555-022-00881-0
  5. Choon SE, Lai NM, Mohammad NA, Nanu NM, Tey KE, Chew SF. 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(6):676-684. doi:10.1111/ijd.12070
  6. Borges-Costa J, Silva R, Gonçalves L, Filipe P, Soares de Almeida L, Marques Gomes M. Clinical and laboratory features in acute generalized pustular psoriasis: a retrospective study of 34 patients. Am J Clin Dermatol. 2011;12(4):271-276. doi:10.2165/11586900-000000000-00000
  7. Knuever J, Tantcheva-Poor I. Generalized pustular psoriasis: a possible association with severe hypocalcaemia due to primary hypoparathyroidism. J Dermatol. 2017;44(12):1416-1417. doi:10.1111/1346-8138.13724
  8. 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
  9. Sano S, Kubo H, Morishima H, Goto R, Zheng R, Nakagawa H. Guselkumab, a human interleukin-23 monoclonal antibody in Japanese patients with generalized pustular psoriasis and erythrodermic psoriasis: efficacy and safety analyses of a 52-week, phase 3, multicenter, open-label study. J Dermatol. 2018;45(5):529-539. doi:10.1111/1346-8138.14294
  10. Imafuku S, Honma M, Okubo Y, et al. Efficacy and safety of secukinumab in patients with generalized pustular psoriasis: a 52-week analysis from phase III open-label multicenter Japanese study. J Dermatol. 2016;43(9):1011-1017. doi:10.1111/1346-8138.13306
  11. Liang J, Huang P, Li H, et al. Mutations in IL36RN are associated with geographic tongue. Hum Genet. 2017;136(2):241-252. doi:10.1007/s00439-016-1750-y
  12. 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
  13. Reisner DV, Johnsson FD, Kotowsky N, Brunette S, Valdecantos W, Eyerich K. Impact of generalized pustular psoriasis from the perspective of people living with the condition: results of an online survey. Am J Clin Dermatol. 2022;23(Suppl 1):65-71. doi:10.1007/s40257-021-00663-y
  14. Lebwohl M, Medeiros RA, Mackey RH, et al. The disease burden of generalized pustular psoriasis: real-world evidence from CorEvitas’ Psoriasis Registry. J Psoriasis Psoriatic Arthritis. 2022;7(2):71-78. doi:10.1177/24755303221079814

Reviewed by Debjyoti Talukdar, MD, on 5/26/2023.

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