Living with GPP - Make the most out of your doctor's appointments
Glossary & FAQs
Glossary
Below is a list of terms often used when discussing GPP, along with their definitions:
A lung injury that allows fluid to leak into the lungs. This is a life-threatening condition, in which breathing becomes difficult and oxygen cannot get into the body.27
An autoimmune disease means that the body's natural defence system can't tell the difference between your own cells and foreign cells (such as viruses and bacteria), causing the body to mistakenly attack healthy cells.28,29
An autoinflammatory disease happens with the innate immune system's reactions. Immune cells target the body's own healthy tissues by mistake, signalling the body to attack them. This can cause intense episodes of inflammation that result in such symptoms as fever, rash, or joint swelling.30
A medical procedure that involves taking a small sample of body tissue so it can be examined under a microscope.31
A symptom consisting of a breakout and becoming visible on the skin.32
An increase in the severity of a disease or its signs and symptoms.33
A skin reaction that can be triggered by an infection or some medicines.34
An outbreak, where symptoms temporarily increase in number or intensity.35
General feeling of discomfort, illness, or lack of well-being.36
The most common type of psoriasis. It usually causes dry, red skin lesions (plaques) covered with silvery scales.37
Small, inflamed, pus-filled, blister-like sores on the skin’s surface.38
Scaling skin is dry, cracked, or flaky skin. Scaling skin happens when the outer layer of the skin, called the epidermis, begins to flake off. A symptom of many medical conditions which can lead to health complications if left untreated.39
The body’s extreme response to an infection. Sepsis happens when an infection you already have triggers a chain reaction throughout your body. It is a life-threatening medical emergency.40
Affect the entire body, rather than a single organ or body part.41
Treatment using substances that travel through the bloodstream, reaching and affecting cells all over the body. Systemic therapies treat the entire body, rather than a particular area, addressing the condition from the inside out.42,43
Treatment focused on the skin surface.44
UV is present in natural sunlight. Ultraviolet light therapy, also known as phototherapy, involves exposing the skin to an artificial UV light source for a set length of time on a regular schedule.45
Frequently asked questions (FAQs)
Expand allGPP is a rare type of psoriasis.1 It is a lifelong, persistent autoinflammatory disease which unfortunately may be life-threatening.6 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
People living with GPP experience episodes or flares of widespread reddened, inflamed skin that's covered with painful pustules. 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 GPP can display with or without plaque psoriasis, and with or without general (systemic) symptoms such as fever, joint pain, or feeling extremely tired.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 not contagious, so you cannot catch it from someone else or pass it on.4 It is an autoinflammatory disease.12
GPP can often be confused with plaque psoriasis, but they are two distinctly different diseases with different symptoms.4 GPP is an autoinflammatory disease, while plaque psoriasis is autoimmune.12 They are caused by different processes in the body and so are treated in different ways.12,17
Check out this video to find out more about the differences between GPP and plaque psoriasis.
GPP flares are defined as episodes 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, and can cause considerable mental and emotional distress.7,8,9
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
If you feel a flare coming on, contact your doctor immediately. When you see or feel the first signs of a flare (even if it’s mild), call your doctor (dermatologist), or go to the hospital. You may require emergency care.
A Centre of Excellence (CoE) for GPP is a health institution where you may be referred for diagnosis or for disease management. The CoEs are made up of either an individual or a group of healthcare professionals that have the strongest level of expertise in dermatology including in GPP.
Download the leaflet to learn more about the CoEs and the referral process.
Your first course of action is to speak to your doctor on whether you need to be referred. They can advise on the process and steps for a referral.
- 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
- 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
- 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
- Pustular Psoriasis [Internet]. National Psoriasis Foundation. 2022 [cited 4 March 2022]. Available from: https://www.psoriasis.org/pustular/
- 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
- 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
- Bachelez H. Pustular psoriasis and related pustular skin diseases. Br J Dermatol. 2018;178(3):614-618. doi: 10.1111/bjd.16232
- Pustular psoriasis [Internet]. The Psoriasis and Psoriatic Arthritis Alliance. 2022 [cited 4 March 2022]. Available from: https://www.papaa.org/
- 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
- Pustular Psoriasis. [Internet]. WebMD.com. 2023 [cited 31 January 2023]. Available from: https://www.webmd.com/skin-problems-and-treatments/psoriasis/pustular-psoriasis
- 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
- 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
- Generalized pustular psoriasis [Internet]. Medlineplus.gov. 2022 [cited 4 March 2022]. Available from: https://medlineplus.gov/genetics/condition/generalized-pustular-psoriasis/
- 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
- 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
- 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
- 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
- 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
- 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
- Benjegerdes K, et al. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis: Targets and Therapy. 2016;6:131-144. doi: 10.2147/PTT.S98954
- 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
- Bachelez H. Pustular Psoriasis: The Dawn of a New Era. Acta Derm Venereol. 2020;100(3):adv00034. doi: 10.2340/00015555-3388
- Pustular Psoriasis [Internet]. eMedicineHealth. 2022 [cited 4 March 2022]. Available from: https://www.emedicinehealth.com/pustular_psoriasis/article_em.htm
- 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
- 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
- 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
- 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
- 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
- Autoimmune Diseases [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/autoimmunediseases.html
- 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
- Biopsy [Internet]. nhs.uk. 2022 [cited 6 May 2022]. Available from: https://www.nhs.uk/conditions/biopsy/
- 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
- Definition of exacerbation [Internet]. Rxlist.com. 2022 [cited 6 May 2022]. Available from: https://www.rxlist.com/exacerbation/definition.htm
- 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
- 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
- Malaise [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/ency/article/003089.htm
- 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
- 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
- Scaling skin: Pictures, causes, treatment, and prevention [Internet]. Medicalnewstoday.com. 2022 [cited 6 May 2022]. Available from: https://www.medicalnewstoday.com/articles/323967#_noHeaderPrefixedContent
- 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
- Systemic [Internet]. Medlineplus.gov. 2022 [cited 6 May 2022]. Available from: https://medlineplus.gov/ency/article/002294.htm
- Systemic therapy [Internet]. National Cancer Institute. 2022 [cited 6 May 2022]. Available from: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/systemic-therapy
- 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
- Using medication: Topical medications [Internet]. Ncbi.nlm.nih.gov. 2022 [cited 6 May 2022]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK361003/
- Phototherapy [Internet]. National Psoriasis Foundation. 2022 [cited 6 May 2022]. Available from: https://www.psoriasis.org/phototherapy/