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Prevalence, comorbidities and mortality of generalized pustular psoriasis: A literature review

Prinz JC, Choon SE, Griffiths CEM, Merola JF, Morita A, Ashcroft DM, et al.

J Eur Acad Dermatol Venereol, 2023 Feb;37(2):256-273.
PMID: 36331364 DOI: 10.1111/jdv.18720

Generalized pustular psoriasis (GPP) is a rare auto-inflammatory skin disease characterised by acute episodes of sterile pustule formation. Diagnosis and treatment of the disease have historically been complicated by a lack of awareness, and no consistent global definition or clinical coding standards. Now acknowledged as a distinct clinical entity with a recognised genetic component, GPP can take a serious and life-threatening course due to systemic inflammatory complications and its association with various comorbidities. As with other rare diseases, there are significant challenges to understanding the epidemiology of GPP, notably a small patient population, non-standardised study methodologies and ethnic differences in its presentation. A clearer understanding of GPP is therefore required for clinicians to better manage patients with this rare condition. In this review article, we present an overview of the available data on GPP prevalence estimates in key demographics and report the frequency of genetic mutations associated with the disease. We detail the incidence of known comorbidities and summarise the data on mortality and assigned causes of death. Lastly, we discuss the various factors that impact the collection, interpretation and comparison of these data.

Matched MeSH terms: Skin Diseases, Vesiculobullous*
Fulltext Clinical Course and Characteristics of Generalized Pustular Psoriasis

Choon SE, Navarini AA, Pinter A

Am J Clin Dermatol, 2022 Jan;23(Suppl 1):21-29.
PMID: 35061227 DOI: 10.1007/s40257-021-00654-z

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening disease characterized by episodes of widespread sterile macroscopic pustules, with or without systemic inflammation and/or plaque psoriasis. Multiple GPP subtypes have been described, from acute GPP of von Zumbusch to milder, annular pustular psoriasis. Generalized pustular psoriasis mainly affects adults, with a female preponderance, but juvenile GPP also occurs. Flares are a hallmark of GPP and may occur de novo or be provoked by triggers, including withdrawal of systemic corticosteroids, infections, stress, pregnancy, and menstruation. Severity of flares varies widely between patients, and between flares in an individual patient. Significant flares are often accompanied by systemic symptoms, notably fever, general malaise, and extracutaneous manifestations such as arthritis, uveitis, and neutrophilic cholangitis. Common laboratory abnormalities include neutrophilia, elevated C-reactive protein levels, hypocalcemia, and abnormal liver function tests. The clinical course of GPP is highly variable; it can be a relapsing disease with recurrent flares and no pustulation between flares or a persistent disease with perpetual mild pustulation punctuated with flares of greater severity. Patients may have multiple flares per year or a flare every few years. Most flares last 2-5 weeks and approximately 50% require hospitalization. Life-threatening complications include sepsis and renal, hepatic, respiratory, and heart failure. Reported mortality rates are 2-16%.

Matched MeSH terms: Skin Diseases, Vesiculobullous/complications*; Skin Diseases, Vesiculobullous/pathology*
Fulltext Clinical Disease Measures in Generalized Pustular Psoriasis

Burden AD, Choon SE, Gottlieb AB, Navarini AA, Warren RB

Am J Clin Dermatol, 2022 Jan;23(Suppl 1):39-50.
PMID: 35061231 DOI: 10.1007/s40257-021-00653-0

Generalized pustular psoriasis (GPP) is a rare neutrophilic skin condition characterized by episodes of widespread eruption of sterile macroscopic pustules that can be associated with systemic inflammation. The rarity of GPP and its heterogeneous cutaneous and extracutaneous symptoms pose considerable challenges to the development and adoption of comprehensive accurate disease measures for the routine clinical assessment of disease severity and the evaluation of new treatments in clinical trials. Psoriasis disease measures remain among the most commonly used methods for evaluating patients with GPP, despite their limitations owing to a lack of assessment of pustules (a hallmark of GPP), systemic inflammation, and disease symptoms. The adaptation of psoriasis disease measures and the development of assessment tools specific for GPP severity will enable more effective and accurate monitoring of patients with GPP and enhance the clinical development of new therapies. Further clinical validation of recently developed modified assessment tools, such as the Generalized Pustular Psoriasis Physician Global Assessment and the Generalized Pustular Psoriasis Area and Severity Index, and international consensus on using quantitative tools and patient-reported outcome measures in the development of new treatments are needed to advance patient care.

Matched MeSH terms: Skin Diseases, Vesiculobullous/complications; Skin Diseases, Vesiculobullous/pathology*
Health-related quality of life in patients with generalized pustular psoriasis: A systematic literature review

Choon SE, De La Cruz C, Wolf P, Jha RK, Fischer KI, Goncalves-Bradley DC, et al.

J Eur Acad Dermatol Venereol, 2024 Feb;38(2):265-280.
PMID: 37750484 DOI: 10.1111/jdv.19530

Generalized pustular psoriasis (GPP) is a rare, chronic, neutrophilic inflammatory skin disease characterized by episodes of widespread eruption of sterile, macroscopic pustules that can be accompanied by systemic inflammation and symptoms. A systematic literature review and narrative synthesis were conducted to determine the impact of GPP on patients' health-related quality of life (HRQoL) and patient-reported severity of symptoms and to compare its impact to patients with plaque psoriasis (plaque PsO). Searches were undertaken in Embase, MEDLINE and the Cochrane Library from 1 January 2002 to 15 September 2022. Screening was carried out by two reviewers independently. Outcome measures included generic (e.g. EQ-5D, SF-36) and dermatology-specific (e.g. DLQI) clinical outcome assessments, and other relevant patient-reported outcome measures (PROMs) (e.g. severity of pain measured by a numerical rating scale). Overall, 20 studies were found to be eligible for inclusion, of which seven also had data for plaque PsO. The DLQI was the most frequently reported outcome measure (16 out of 20 studies). When reported, mean DLQI (SD) scores varied from 5.7 (1.2) to 15.8 (9.6) across the studies, indicating a moderate to very large effect on HRQoL; the wide range of scores and large SDs were explained by the small population sizes (n ≤ 12 for all studies except two). Similar ranges and large SDs were also observed for other measures within individual studies. However, in general, people with GPP reported a greater impact of their skin condition on HRQoL, when compared to people with plaque PsO (i.e. higher DLQI scores) and higher severity for itch, pain and fatigue. This systematic review highlighted the need for studies with a larger population size, a better understanding of the impact of cutaneous and extracutaneous symptoms and comorbidities on HRQoL during and between GPP flares, and outcome measures specifically tailored to the unique symptoms and the natural course/history of GPP.

Matched MeSH terms: Skin Diseases, Vesiculobullous*
Bullous contact allergy from cinnamon

Goh CL, Ng SK

Derm Beruf Umwelt, 1988 Nov-Dec;36(6):186-7.
PMID: 3069433

A young Malay female presented with a bullous eruption as a manifestation of contact allergy to cinnamic aldehyde in cinnamon. The clinical and histological features resembled bullous pemphigoid but immunological markers for pemphigoid were absent. The patient responded to a short course of oral steroids. Contact allergy can mimic pemphigoid.

Matched MeSH terms: Skin Diseases, Vesiculobullous/etiology*
Bullous diseases: a 7-year survey of experience with 77 patients

Adam BA

Ann Acad Med Singap, 1983 Jan;12(1):19-25.
PMID: 6344741

A prospective study of 77 consecutive patients with bullous diseases was done to study the pattern and natural history. Pemphigus was the commonest with 45 patients (59%) followed by pemphigoid with 21 patients (27%). Pemphigoid was more common in Indians than in other ethnic groups and its age of onset was a decade later than pemphigus. Unusual immunofluorescent findings in both diseases are discussed. Six of the 7 patients with dermatitis herpetiformis had linear IgA in the dermo-epidermal junction and the classical papillary IgA deposits were absent. Ultrastructural findings of pemphigoid and dermatitis herpetiformis confirmed earlier reports. Chronic bullous dermatoses of childhood was seen in 4 patients, all of whom had total remission within one year of onset disease.

Matched MeSH terms: Skin Diseases, Vesiculobullous/immunology*; Skin Diseases, Vesiculobullous/epidemiology
Fulltext Can Salmonella enteritidis cause fatal necrotising fasciitis? A case report

Dhanoa A, Singh VA

BMJ Case Rep, 2009;2009.
PMID: 21686336 DOI: 10.1136/bcr.12.2008.1401

This is a presentation of a case of mono microbial necrotising fasciitis due to the unusual organism Salmonella enteritidis. The patient presented with swelling and blistering of the right calf. There are only five other such cases reported in the literature. This was the only case that had positive blood cultures for the organism. Prompt and appropriate treatment was intuited but the patient died because of multi-organ failure.

Matched MeSH terms: Skin Diseases, Vesiculobullous
Fulltext Childhood Occurrence of Pemphigus

U Patil R, T Anegundi R, R Gujjar K, Indushekar KR

Int J Clin Pediatr Dent, 2017 06 01;10(2):196-200.
PMID: 28890623 DOI: 10.5005/jp-journals-10005-1434

Pemphigus is a chronic mucocutaneous disease that initially manifests in the form of intraoral blisters which spread to other mucous membrane and skin. This study describes an unusual case of chronic generalized childhood pemphigus disease in an 11-year-old girl, who presented with multiple vesicles all over her body. Such a condition is seen more often in older people rather than children. It is crucial for dental professionals to be familiar with the diagnosis of bullous skin diseases in children and adolescents, especially in its initial stages in order to prevent the serious consequences and morbidity. The article highlights clinical presentation, histopathology, and successful management strategies useful for pediatric dental practice.
HOW TO CITE THIS ARTICLE: Patil RU, Anegundi RT, Gujjar KR, Indushekar KR. Childhood Occurrence of Pemphigus. Int J Clin Pediatr Dent 2017;10(2):196-200.

Matched MeSH terms: Skin Diseases, Vesiculobullous
Incidence and prevalence of generalized pustular psoriasis in multiethnic Johor Bahru, Malaysia: a population-based cohort study using routinely captured electronic health records in the Teleprimary Care (TPC®) clinical information system from 2010 to 2020

Choon SE, Wright AK, Griffiths CEM, Wong KW, Tey KE, Lim YT, et al.

Br J Dermatol, 2023 Sep 15;189(4):410-418.
PMID: 37162007 DOI: 10.1093/bjd/ljad158

BACKGROUND: There is limited understanding of the epidemiology of generalized pustular psoriasis (GPP) internationally, with no population-based estimates of GPP in South East Asia.
OBJECTIVES: To determine the incidence and prevalence of GPP in the Malaysian population and characterize its flares and trigger factors.
METHODS: We conducted a population-based cohort study using the Teleprimary Care database between January 2010 and December 2020. We identified 230 dermatologist-confirmed GPP cases using International Classification of Diseases, 10th revision, diagnostic codes. Annual prevalence and incidence rates were stratified by age, sex and ethnicity. We compared data regarding flares and trigger factors for patients with GPP who had associated psoriasis vulgaris (PV) with those who did not have associated PV.
RESULTS: The prevalence of GPP was 198 per million (267 women, 127 men) and incidence was 27.2 per million person-years [95% confidence interval (CI) 22.8-31.6]; 35.3 (28.4-42.2) per million person-years for women and 18.3 (13.1-23.5) per million person-years for men. Rates were higher in Chinese individuals [prevalence 271 per million; incidence 41.6 per million person-years (28.9-54.3)] than in the Malay population [prevalence 186; incidence 24.6 (19.4-29.7)] or the Indian ethnic group [prevalence 179; incidence 25.0 (13.8-36.3)]. Annual prevalence was consistently higher in women than in men and highest among the Chinese population, followed by the Indian and Malay populations. Overall, 67% of patients with GPP had associated PV. The prevalence and incidence of GPP without PV were lower than GPP with PV at 66 vs. 132 per million and 19.3 (95% CI 15.6-23.0) vs. 8.0 (95% CI 5.6-10.3) per million person-years, respectively. The mean age at GPP onset was 42.7 years (SD 18.4). A bimodal trend in the age of GPP onset was observed, with first and second peaks at age 20-29 years and age 50-59 years, respectively. Disease onset was significantly earlier in patients with GPP without PV than in those with PV [mean age 37.5 years (SD 20.7) vs. 44.9 years (SD 17.0), P = 0.026]. Flares occurred more frequently in patients without PV than in those with PV [mean number of flares per patient per year was 1.35 (SD 0.77) vs. 1.25 (SD 0.58), P = 0.039]. Common triggers of flares in patients with GPP who did not have PV were infections, pregnancy, menstruation and stress, whereas withdrawal of therapy, particularly systemic corticosteroids, was a more frequent trigger in patients with GPP who also had PV.
CONCLUSIONS: Our findings contribute to the global mapping of GPP, which will help inform the management of this rare condition.

Matched MeSH terms: Skin Diseases, Vesiculobullous*
Fulltext A case of pustular psoriasis in a young boy

Meera Kuppusamy, Tarita Taib

Journal of Clinical and Health Sciences, 2017;2:39-42.

We report a case of a 13-year-old boy who presented with acute onset of generalised erythematous skin and patchy areas of pustules for one week duration. He was well until one month ago when he started having small scaly plaques on his scalp and extensors of his legs. During the acute episode, he also had joint pain and bilateral conjunctivitis. Skin biopsy confirmed pustular psoriasis. He developed leucocytosis and transaminitis during the acute phase of the pustular eruption while on acitretin, which was then withheld. Subsequently, treatment with oral cyclosporine induced remission of his skin and joint disease. The case is hereby reported because of rarity of presentation and clinical features. Oral cyclosporine should be considered in patients with generalised pustular psoriasis complicated with transaminitis.

Matched MeSH terms: Skin Diseases, Vesiculobullous
Fulltext A case of pustular psoriasis in a young boy

Meera Kuppusamy, Tarita Taib

Journal of Clinical and Health Sciences, 2017;2(1):39-42.
MyJurnal

We report a case of a 13-year-old boy who presented with acute onset of generalised erythematous skin and patchy areas of pustules for one week duration. He was well until one month ago when he started having small scaly plaques on his scalp and extensors of his legs. During the acute episode, he also had joint pain and bilateral conjunctivitis. Skin biopsy confirmed pustular psoriasis. He developed leucocytosis and transaminitis during the acute phase of the pustular eruption while on acitretin, which was then withheld. Subsequently, treatment with oral cyclosporine induced remission of his skin and joint disease. The case is hereby reported because of rarity of presentation and clinical features. Oral cyclosporine should be considered in patients with generalised pustular psoriasis complicated with transaminitis.

Matched MeSH terms: Skin Diseases, Vesiculobullous
Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia

Lau BW, Lim DZ, Capon F, Barker JN, Choon SE

Int J Dermatol, 2017 Apr;56(4):392-399.
PMID: 28194751 DOI: 10.1111/ijd.13489

BACKGROUND: Limited information exists regarding juvenile generalized pustular psoriasis (GPP). We aim to determine the clinical profile and outcome of Malaysians with juvenile GPP.
METHODS: Review of hospital case notes on patients with juvenile GPP.
RESULTS: Twenty-seven patients with juvenile GPP were identified. Female to male ratio was 1.4:1. The median age at onset of GPP was 6.5 years. Ten patients had prior psoriasis with a median pre-pustular duration of 2.7 years. Onset of GPP was earlier in patients without prior psoriasis (5.1 years vs. 12.0 years, P = 0.002). Precipitating factors identified included stress, upper respiratory tract infection, systemic steroid use, vaccination, and pregnancy. A positive family history of psoriasis and GPP was present in six and one patient(s), respectively. Twenty-one patients had acute, five annular, and one localized variant of GPP. Arthritis was present in 22.2%. Fever, leukocytosis, and transaminitis were mainly seen in patients with acute GPP at 80.9, 72.2, and 11.1%, respectively. Among 20 patients screened, eight carry IL36RN variants and one has CARD14 mutation. IL36RN-positive patients have more severe disease characterized by early onset, low prevalence of prior plaque psoriasis, high prevalence of systemic inflammation, and need for continuous long-term systemic therapy. Acitretin and cyclosporine were effective in aborting acute GPP in 100% of 16 and 66.7% of six patients treated, respectively. However, relapses were common. Only three of the 17 patients whose initial acute GPP was controlled with systemic agents were successfully weaned off treatment.
CONCLUSIONS: Juvenile GPP is a chronic recalcitrant disease. IL36RN-positive patients have more severe disease.

Matched MeSH terms: Skin Diseases, Vesiculobullous

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