The chronic nature of generalized pustular psoriasis

Chronicity is a key feature of generalized pustular psoriasis (GPP)

GPP is a rare disease that varies widely between patients; however, one characteristic remains the same – its chronicity. The chronicity of GPP is defined by persistent skin inflammation and periodic flare-ups of severe intensity. It can be hard for patients to complete everyday tasks with the ongoing symptoms. Approximately 50% of people experiencing a GPP flare may require hospitalization.²

Chronic skin symptoms^{2,3,4 ,5 ,6 ,7} 

• Common symptoms include persistent scaling, crusting and/or erythema between flares
• They may occur with or without systemic symptoms
• Defined as maintained over the course of more than 3 months

Periodic flare-ups^{2,4 ,5} 

• Are characterized by a widespread eruption of pustules, erythema, and scaling
• May occur with or without systemic inflammation
• Can vary in intensity, duration and frequency
• The majority of flares last between 2 and 5 weeks
• Some people could have numerous flares per year, while others could have a flare every few years

Physical and emotional symptoms of GPP

GPP can take a daily toll, with people living with the disease struggling with the unpredictability of the condition.^3,5, 
People with GPP tend to have higher rates of anxiety and depression compared to those with plaque psoriasis.  Recent data shows: ^{5, 10}

*Dermatologists in the CorEvitas (previously Corrona) Psoriasis Registry (a collaboration with the National Psoriasis Foundation) who had treated adult patients (aged ≥18 years) with GPP within the past 5 years (N=29).⁵
†Based on a retrospective review of 102 patients with adult-onset GPP in a tertiary hospital in Malaysia.¹⁰
‡Chronic symptoms of GPP included pustular lesions (n=22) and multiple ill-defined erythematous thin plaques (n=10). The specific type of systemic treatment used after a flare was not reported, but treatment of initial GPP flares included retinoids, methotrexate, cyclosporin, adalimumab, doxycycline, dapsone and prednisolone.¹⁰
 

Ongoing physical symptoms of GPP:

Emotional distress:

Patient-centric approach to care

Ongoing care is common even after a flare has resolved.⁵ Holistic care plans offer more integrated support for people living with GPP. It’s important that healthcare professionals and patients work together when building care plans, aiming to alleviate the daily burden of GPP and improve quality of life. This way, those with the condition can gain confidence to get back to doing the things they love in life. 
 

References

1. Takeichi T, Akiyama M. Generalized pustular psoriasis: clinical management and update on auto inflammatory aspects. Am J Clin Dermatol. 2020;21(2):227–236.
    
2. Rivera-Diaz R, Daudén E, Carrascosa JM, de la Cueva P, Puig L. Generalized pustular psoriasis: a review on clinical characteristics, diagnosis, and treatment. Dermatol Ther (Heidelb). 2023;13(3):673–688.
    
3. Reisner, DV, Johnsson FD, Kotowsky, N, et al. 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):61–71.
    
4. 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.
    
5.  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.
    
6.  Choon SE, Navarini AA, Pinter A. Clinical course and characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):21–29.
    
7.  Puig L, Choon SE, Gottlieb AB, et al. Generalized pustular psoriasis: A global Delphi consensus on clinical course, diagnosis, treatment goals and disease management. J Eur Acad Dermatol Venereol. 2023;37(4):737–752.
    
8.  Lebwohl M, Medeiros, RA, Mackey RH, et al. The disease burden of generalized pustular psoriasis: real-world evidence from the CorEvitas’ Psoriasis Registry. J Psoriasis Psoriatic Arthritis. 2022;7(2):71–78.
    
9.  Kotowsky N, Feldman S, Garry EM, Valdecantos WC, Gao R, Golembesky AK. Characteristics of patients with generalized pustular psoriasis compared with those with psoriasis vulgaris: a claims database study. Poster presented at: 16th Annual Maui Derm for Dermatologists; January 25–29, 2020. Maui, Hawaii.
    
10. 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.
     
11. Shah M, Al Aboud DM, Crane JS, Kumar S. Pustular psoriasis. StatPearls. Updated August 8, 2022. Available from: www.ncbi.nlm.nih.gov/books/NBK537002 . Accessed April 2024.