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Recognizing GPP flares

RECOGNIZING GPP FLARES

IDENTIFY THE CHARACTERISTICS OF GPP FLARES

Generalized pustular psoriasis (GPP) flares are characterized by episodes of eruptions of neutrophilic pustules and worsening lesions.1 These episodes can be recurring or persistent for patients.2 Symptoms of GPP can occur with or without systemic inflammation and with or without plaque psoriasis.3

 

HOW GPP FLARES CAN MANIFEST ON PATIENTS' SKIN

The most-cited GPP symptoms include pustules and worsening lesions.1

gpp pustules

 

Image used with permission from Dr Siew Eng Choon.

gpp pustules 2

 

Image used with permission from Dr Mahira Hamdy El Sayed.

OTHER SYMPTOMS MAY INCLUDE1,4-6:

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ITCHING
scaling icon

SCALING
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DRYNESS
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PAIN
swelling icon

SWELLING
burning sensation icon

BURNING SENSATION
erythema icon

ERYTHEMA
fever icon

FEVER
leukocytosis icon

LEUKOCYTOSIS
joint pain icon

JOINT PAIN
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FATIGUE
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HEADACHE

WATCH THE VIDEO FOR MORE ABOUT GPP FLARES

GPP FLARES MAY REQUIRE SAME-DAY MEDICAL ASSESSMENT7

There are serious complications associated with GPP to consider. These include acute respiratory distress syndrome (ARDS), sepsis, cardiovascular aseptic shock, congestive heart failure, renal failure, neutrophilic cholangitis, metabolic abnormalities, osteoarthritis, and uveitis. The most common causes of death associated with GPP are sepsis, ARDS, and cardiac failure.4,8-10

THE UNPREDICTABLE NATURE OF FLARES MAKES GPP CHALLENGING TO CONTROL4

GPP flares can recur and vary among patients and even present differently within the same patient.2,4 Because of this, previous flare episodes do not indicate the severity or duration of the next flare.4 Due to the significant burden of disease, it is important to stabilize patients to prevent escalation.1,9,10

LEFT UNTREATED, GPP SYMPTOMS CAN ESCALATE AND MAY RESULT IN HOSPITALIZATION AND BECOME LIFE-THREATENING1,8

GPP can affect patients’ quality of life. It can impact patients’ social relationships and mental health, and is associated with higher rates of anxiety and depression disorders.1,5,10-12

SURVEYED DERMATOLOGISTS HAVE REPORTED THAT THE DURATION OF GPP FLARES AND TIME TO RESOLUTION MAY VARY1

To better characterize GPP, the Corrona Psoriasis Registry conducted a survey of dermatologists in the US and Canada. These dermatologists were identified as likely to be currently treating, or had treated adult patients (age ≥ 18 years) with GPP within the past 5 years. The findings of this survey were published in April 2021.

average length of flares and average time to resolution charts

CONSIDER HOW GPP FLARES CAN AFFECT PATIENTS10,13

Severe GPP symptoms can make it difficult for patients to complete daily activities.10 A recent study using the CorEvitas Psoriasis Registry showed that median patient-reported pain, itching, and fatigue were higher with GPP than with plaque psoriasis.13

patient-reported symptom measures chart

 

SEVERAL UNMET NEEDS EXIST IN THE DIAGNOSIS AND TREATMENT OF GPP1,14

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GPP IS A RARE, CHRONIC DISEASE THAT PHYSICIANS SELDOM SEE, WHICH MAY HINDER PHYSICIAN CONFIDENCE IN DIAGNOSIS.15,16

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PATIENTS MAY PRESENT IN THE EMERGENCY SETTING, WHERE THERE MAY NOT BE A DERMATOLOGIST ON CALL.17

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THERE ARE CURRENTLY NO DIAGNOSTIC OR PROGNOSTIC BIOMARKERS FOR GPP.18

AN EFFECTIVE THERAPY SHOULD:

  • Rapidly and effectively resolve GPP flares1,10
  • Have an acceptable safety profile10,19
  • Provide sustained disease control1

Artwork representing the experience of patients with GPP

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REFERENCES

  1. Strober B, Kotowsky N, Medeiros R, 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

  2. 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

  3. Navarini AA, Burden AD, Capon F, et al; for the 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

  4. Bachelez H. Pustular psoriasis and related pustular skin diseases. Br J Dermatol. 2018;178(3):614-618. doi:10.1111/bjd.16232

  5. Skalicky A, Rentz A, Esser D, Thoma C, Gloede T. Symptom experience of patients with generalized pustular psoriasis (GPP). Value in Health. 2020;23(suppl 1):S345. Abstract: PRO89. doi:10.1016/j.jval.2020.04.1310

  6. Shah M, Al Aboud DM, Crane JS, Kumar S. Pustular psoriasis. StatPearls. Updated August 10, 2020. Accessed October 20, 2021. www.ncbi.nlm.nih.gov/books/NBK537002/

  7. Psoriasis: assessment and management. NICE Clinical Guideline [CG153]. National Institute for Health and Care Excellence. Published October 24, 2012. Updated September 2017. Accessed October 20, 2021. www.nice.org.uk/guidance/cg153

  8. Ly K, Beck KM, Smith MP, Thibodeaux Q, Bhutani T. Diagnosis and screening of patients with generalized pustular psoriasis. Psoriasis (Auckl). 2019;9:37-42. doi:10.2147/PTT.S181808

  9. 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

  10. Kharawala S, Golembesky AK, Bohn RL, Esser D. The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review. Expert Rev Clin Immunol. 2020;16(3):239-252. doi:10.1080/1744666X.2019.1708193

  11. Sampogna F, Tabolli S, Söderfeldt B, Axtelius B, Aparo U, Abeni D; for the IMPROVE Investigators. Measuring quality of life of patients with different clinical types of psoriasis using the SF-36. Br J Dermatol. 2006;154(5):844-849. doi:10.1111/j.1365-2133.2005.07071.x

  12. Kotowsky N, Gao R, Singer D, Garry E, Golembesky AK. Healthcare resource utilization (HCRU) in patients with generalized pustular psoriasis (GPP): a claims database study. Value in Health. 2020;23(suppl 1):S333-S334. Abstract: PRO29. doi:10.1016/j.jval.2020.04.1257

  13. 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

  14. Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis (Auckl). 2016;6:131-144. doi:10.2147/PTT.S98954

  15. 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(Suppl1):65-71. doi:10.1007/s40257-021-00663-y

  16. Akiyama M, Takeichi T, McGrath JA, Sugiura K. Autoinflammatory keratinization diseases: an emerging concept encompassing various inflammatory keratinization disorders of the skin. J Dermatol Sci. 2018;90(2):105-111. doi:10.1016/j.jdermsci.2018.01.012

  17. Strober B, Leman J, Mockenhaupt M. 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

  18. 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

  19. 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