Living with GPP - Make the most out of your doctor's appointments

Questions to ask your doctor

middle aged woman, concerns around GPP, understanding GPP, Act4GPP

Understanding GPP

What is GPP?

If you or someone you know has been diagnosed with Generalised Pustular Psoriasis (GPP), it’s important to know what GPP is.

GPP is a rare type of psoriasis.1 It is a lifelong, persistent autoinflammatory disease which unfortunately may be life-threatening.2 If left untreated, it can lead to complications including sepsis (infection), kidney failure, heart failure, and acute respiratory distress syndrome (fluid in the lungs).3

GPP is not contagious, so you cannot catch it from someone else or pass it on.4 Because GPP is so rare, many people (including doctors) are not familiar with it, meaning it’s not easy to recognise.5 In fact, patients with GPP may be wrongly diagnosed, as it can be mistaken as an infection or other diseases.6

With GPP, exacerbations or flares appear suddenly in the form of painful pustules over large areas of the skin.7 These are red, itchy, painful, and highly visible to other people. They may be accompanied by fever, chills, fatigue and nausea, as well as considerable mental and emotional distress.7-9 Pustules are inflamed, blister-like sores (lesions) that are filled with pus that appear on the skin’s surface, which can be scaly, flaky or itchy and can also be painful.10,11 People living with GPP experience pustules which often expand and combine, lasting from days to weeks.12 GPP can be persistent, with symptoms lasting for months, or relapsing, with symptoms resolving and then reappearing.3

Unfortunately, it’s not known exactly what causes GPP. Some people are naturally prone to getting it, but it can also be triggered by things like stress, certain medications, or pregnancy.7

GPP is a serious condition which requires systemic treatment (that works throughout the whole body), and not just topical treatment.6

Want to learn more about GPP?

Information on GPP

While GPP is rare, here are some things we already know about the disease:

GPP is not contagious, you cant catch it from someone else.  Act4GPP

GPP is not contagious.4 It is an autoinflammatory disease so you can’t catch it from or give it to someone else.12

patients living with GPP, people affected in Europe. Act4GPP

GPP is estimated to affect 2 per million people in Europe.13

40-50 years of age, people living with GPP. Act4GPP

GPP often first appears between 40-50 years of age.1

twice as many women living with GPP, gender. Act4GPP

GPP impacts twice as many women as men.9

type of psoriasis, difference between GPP and PP. Act4GPP

GPP is a specific type of psoriasis, not like plaque psoriasis.3

with and without associated psoriasis, Act4GPP

GPP can occur with or without associated psoriasis.14

life threatening complications, sepsis, organ failure, emergency care, Act4GPP

If left untreated, GPP can lead to life-threatening complications (such as sepsis or organ failure) often requiring emergency care.3

dermatologist, diagnosis of GPP, specialist, Act4GPP

A dermatologist is the right specialist to diagnose GPP.15

Check out this video
to understand more about GPP

  1. 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(6):676-684. doi:10.1111/ijd.12070
  2. Strober B, et al. Unmet medical needs in the treatment and management of generalized pustular psoriasis flares: evidence from a survey of Corrona registry dermatologists. Dermatol Ther (Heidelb). 2021;11(2):529-541. doi:10.1007/s13555-021-00493-0
  3. Ly K, et al. Diagnosis and screening of patients with generalized pustular psoriasis. Psoriasis (Auckl). 2019;20;9:37-42. doi: 10.2147/PTT.S181808
  4. Pustular Psoriasis [Internet]. National Psoriasis Foundation. 2022 [cited 4 March 2022]. Available from: https://www.psoriasis.org/pustular/
  5. Global Commission to End the Diagnostic Odyssey for Children with a Rare Disease [Internet]. Global Commission. 2022 [cited 4 March 2022]. Available from: https://www.globalrarediseasecommission.com/report
  6. Strober B, Leman J, Mockenhaupt M, Nakano de Melo J, Nassar A, Prajapati VH, Romanelli P, Seneschal J, Tsianakas A, Wei LY, Yasuda M, Yu N, Hernandez Daly AC, Okubo Y. Unmet Educational Needs and Clinical Practice Gaps in the Management of Generalized Pustular Psoriasis: Global Perspectives from the Front Line. Dermatol Ther (Heidelb). 2022;12(2):381-393. doi: 10.1007/s13555-021-00661-2
  7. Bachelez H. Pustular psoriasis and related pustular skin diseases. Br J Dermatol. 2018;178(3):614-618. doi: 10.1111/bjd.16232
  8. Pustular psoriasis [Internet]. The Psoriasis and Psoriatic Arthritis Alliance. 2022 [cited 4 March 2022]. Available from: https://www.papaa.org/
  9. Sampogna F, et al. IDI Multipurpose Psoriasis Research on Vital Experiences (IMPROVE) investigators. Measuring quality of life of patients with different clinical types of psoriasis using the SF-36. Br J Dermatol. 2006;154(5):844-9. doi: 10.1111/j
  10. Pustular Psoriasis. [Internet]. WebMD.com. 2023 [cited 31 January 2023]. Available from: https://www.webmd.com/skin-problems-and-treatments/psoriasis/pustular-psoriasis
  11.  Pustules [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/ency/article/003234.htm#:~:text=Pustules%20are%20small%2C%20inflamed%2C%20pus,lesions)%20on%20the%20skin%20surface
  12. Gooderham MJ, et al. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 2019;15(9):907-919. doi: 10.1080/1744666X.2019.1648209
  13. Generalized pustular psoriasis [Internet]. Medlineplus.gov. 2022 [cited 4 March 2022]. Available from: https://medlineplus.gov/genetics/condition/generalized-pustular-psoriasis/
  14. Navarini AA, et al. ERASPEN Network. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017;31(11):1792-1799. doi: 10.1111/jdv.14386
  15. Pustular psoriasis: treatment options. [Internet]. American Academy of Dermatology. 2022 [cited 10 March 2022]. Available from: https://www.aad.org/public/diseases/psoriasis/treatment/genitals/pustular
  16. Skalicky A, et al. Symptom experience of patients with generalized pustular psoriasis (GPP). Value in Health. 2020;23(suppl 1):S345. doi:10.1016/j.jval.2020.04.1310
  17. Johnston A, et al. IL-1 and IL-36 are dominant cytokines in generalized pustular psoriasis. J Allergy Clin Immunol. 2017;140(1):109-120. doi: 10.1016/j.jaci.2016.08.056
  18. Robinson A, et al. Treatment of pustular psoriasis: From the Medical Board of the National Psoriasis Foundation. Journal of the American Academy of Dermatology. 2012;67(2):279-288. doi: 10.1016/j.jaad.2011.01.032
  19. Liang Y, et al. Psoriasis: a mixed autoimmune and autoinflammatory disease. Curr Opin Immunol. 2017;49:1-8. doi:10.1016/j.coi.2017.07.007
  20. Benjegerdes K, et al. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis: Targets and Therapy. 2016;6:131-144. doi: 10.2147/PTT.S98954
  21. Choon SE, et al. 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
  22. Bachelez H. Pustular Psoriasis: The Dawn of a New Era. Acta Derm Venereol. 2020;100(3):adv00034. doi: 10.2340/00015555-3388
  23. Pustular Psoriasis [Internet]. eMedicineHealth. 2022 [cited 4 March 2022]. Available from: https://www.emedicinehealth.com/pustular_psoriasis/article_em.htm
  24. ClinicalTrials.gov. [Internet] A 5-year Study to Test BI 655130 in Patients With Generalized Pustular Psoriasis Who Took Part in Previous Studies With BI 655130 - Full Text View - Clinicaltrials.gov. 2022 [cited 5 April 2022]. Available from: https://www.clinicaltrials.gov/ct2/show/NCT03886246?cond=Generalised+Pustular+Psoriasis&draw=2&rank=5
  25. Sampogna F, et al. Measuring quality of life of patients with different clinical types of psoriasis using the SF-36. British Journal of Dermatology. 2006;154(5):844-849. doi: 10.1111/j.1365-2133.2005.07071.x
  26. Pfohler C, et al. Psoriasis vulgaris and Psoriasis pustulosa; Epidemiology, Quality of Life, Comorbidities and Treatment. Current Rheumatology Reviews. 2013;9(1):2-7. doi: 10.2174/1573397111309010002
  27. Acute Respiratory Distress Syndrome (ARDS) [Internet]. Lung.org. 2022 [cited 6 May 2022]. Available from: https://www.lung.org/lung-health-diseases/lung-disease-lookup/ards
  28.  What Are Common Symptoms of Autoimmune Disease? [Internet]. Hopkinsmedicine.org. 2022 [cited 6 May 2022]. Available from: https://www.hopkinsmedicine.org/health/wellness-and-prevention/what-are-common-symptoms-of-autoimmune-disease
  29. Autoimmune Diseases [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/autoimmunediseases.html
  30. Autoimmune Diseases May Have Features of both Autoimmune and Autoinflammatory Diseases - ENTtoday [Internet]. ENTtoday. 2022 [cited 6 May 2022]. Available from: https://www.enttoday.org/article/autoimmune-diseases-may-have-features-of-both-autoimmune-and-autoinflammatory-diseases/?singlepage=1&theme=print-friendly
  31. Biopsy [Internet]. nhs.uk. 2022 [cited 6 May 2022]. Available from: https://www.nhs.uk/conditions/biopsy/
  32. Eruption [Internet]. Tabers.com. 2022 [cited 10 June 2022]. Available from: https://www.tabers.com/tabersonline/view/Tabers-Dictionary/731677/all/eruption#:~:text=1.,the%20skin%20or%20mucous%20membrane
  33. Definition of exacerbation [Internet]. Rxlist.com. 2022 [cited 6 May 2022]. Available from: https://www.rxlist.com/exacerbation/definition.htm
  34. Erythema [Internet]. Drugwatch.com. 2022 [cited 6 May 2022]. Available from: https://www.drugwatch.com/health/rash-and-skin-disorders/erythema/#:~:text=Erythema%20is%20a%20type%20of,from%20mild%20to%20life%20threatening
  35. How to recognise Fibromyalgia flares [Internet]. Medicalnewstoday.com. 2022 [cited 10 June 2022]. Available from: https://www.medicalnewstoday.com/articles/315646#what-is-a-fibromyalgia-flareup
  36.  Malaise [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/ency/article/003089.htm
  37. Psoriasis - Symptoms and causes [Internet]. Mayo Clinic. 2022 [cited 6 May 2022]. Available from: https://www.mayoclinic.org/diseases-conditions/psoriasis/symptoms-causes/syc-20355840#:~:text=Plaque%20psoriasis%20is%20the%20most,plaques)%20covered%20with%20silvery%20scales
  38. Pustules [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/ency/article/003234.htm#:~:text=Pustules%20are%20small%2C%20inflamed%2C%20pus,lesions)%20on%20the%20skin%20surface
  39. Scaling skin: Pictures, causes, treatment, and prevention [Internet]. Medicalnewstoday.com. 2022 [cited 6 May 2022]. Available from: https://www.medicalnewstoday.com/articles/323967#_noHeaderPrefixedContent
  40. What is sepsis [Internet]. Centers for Disease Control and Prevention. 2022 [cited 6 May 2022]. Available from: https://www.cdc.gov/sepsis/what-is-sepsis.html
  41. Systemic [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/ency/article/002294.htm
  42. Systemic therapy [Internet]. National Cancer Institute. 2022 [cited 6 May 2022]. Available from: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/systemic-therapy
  43. Systemic vs. topical psoriasis treatments: What to know [Internet]. Medicalnewstoday.com. 2022 [cited 6 May 2022]. Available from: https://www.medicalnewstoday.com/articles/btc-systemic-vs-topical-psoriasis-treatments
  44. Using medication: Topical medications [Internet]. Ncbi.nlm.nih.gov. 2022 [cited 6 May 2022]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK361003/
  45. Phototherapy [Internet]. National Psoriasis Foundation. 2022 [cited 6 May 2022]. Available from: https://www.psoriasis.org/phototherapy/