Epidemiological survey of patients with pustular psoriasis in the Japanese Society for Psoriasis Research from 2017 to 2020

Ohtsuki, Mamitaro

doi:10.1111/1346-8138.16583

Cited by 10 publications

(13 citation statements)

References 29 publications

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“…reported stress, infection and certain drugs as the most common triggers for exacerbations of pustular psoriasis in patients in Japan. 31 GPP flares also occur without a known trigger. Idiopathic flares have been reported in 15% of patients by Choon et al and 57% of patients by Zheng et al 5,29 Even when initial flares have known triggers, subsequent flares can occur without known causes.…”

Section: G Pp Patient Burdenmentioning

confidence: 99%

“…The same study reported 58.4% of patients received oral medication, 44.0% received biologics and 9.6% received phototherapy. 31 In another retrospective analysis of hospitals in Japan, 1516 patients with GPP were identified that were treated with biologics (19%), oral agents (63%) and corticosteroids only (18%). 16 Patients with GPP treated with biologics had significantly less in-hospital morbidity (5.4% biologics vs. 8.2% oral agents vs. 12% corticosteroids only, p = 0.02), respiratory complications (1.7% vs. 3.7% vs. 8.8%, p < 0.001), and mortality (1.0% vs. 3.7% vs. 9.1%, p < 0.001).…”

Section: Treatment Utilizationmentioning

confidence: 99%

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A narrative review of the socioeconomic burden associated with generalised pustular psoriasis

Zimmermann

Hofmann

Chiu

2023

Experimental Dermatology

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Generalised pustular psoriasis (GPP) is a rare, heterogeneous, chronic and potentially life-threatening autoinflammatory skin disease, characterised by episodes of widespread eruption of sterile, macroscopic pustules that occur with or without symptoms and signs of systemic inflammation. 1,2 The aberrant activation of the interleukin-36 pathway in patients with GPP causes an inflammatory reaction and widespread eruption of pustules. Flares, the acute phase of GPP involving widespread pustules, skin lesions and erythema, 3 typically last 2-5 weeks, 4 but may persist longer than 3 months and may be accompanied by systemic symptoms such as fever, pain, and fatigue. [5][6][7] While the most dominant cutaneous symptoms are pustules, erythema and scaling,

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Section: G Pp Patient Burdenmentioning

confidence: 99%

Section: Treatment Utilizationmentioning

confidence: 99%

A narrative review of the socioeconomic burden associated with generalised pustular psoriasis

Zimmermann

Hofmann

Chiu

2023

Experimental Dermatology

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“…It accounts for only 0.6%-13.1% of all childhood psoriasis. [1][2][3] The loss-of-function mutation of interleukin-36 receptor antagonist (IL36Ra) has been associated with development of GPP. 4 Interestingly, IL-17 mRNA levels were also noted to be higher in pustular psoriasis in comparison with other clinical subtypes.…”

Section: E T T E R T O T H E E D I T O R Infantile Generalized Pustul...mentioning

confidence: 99%

Infantile generalized pustular psoriasis with excellent response to secukinumab: A case report

Guevara¹,

Aquino²,

Diaz³

et al. 2023

Experimental Dermatology

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“…A range of factors have been identified as triggers of GPP, including pregnancy, infections, and starting or withdrawing treatments such as systemic corticosteroids or anti‐psoriatic therapy 2,6,7,9,10 . Additionally, psoriasis vulgaris (PsV) may precede GPP, with 34%–65% of patients with GPP having prior or concomitant PsV 2,6,9–11 ; it was recently shown that, in Japan, 29.7% of patients with GPP have prior or concomitant PsV 12 . PsV is a papulosquamous skin disease characterized by acanthosis, parakeratosis, and dermal inflammatory infiltrates 2,13 .…”

Section: Introductionmentioning

confidence: 99%

“…2,6,7,9,10 Additionally, psoriasis vulgaris (PsV) may precede GPP, with 34%-65% of patients with GPP having prior or concomitant PsV 2,6,[9][10][11] ; it was recently shown that, in Japan, 29.7% of patients with GPP have prior or concomitant PsV. 12 PsV is a papulosquamous skin disease characterized by acanthosis, parakeratosis, and dermal inflammatory infiltrates. 2,13 PsV can present with a few scattered red, scaly plaques or can involve a greater body surface area.…”

mentioning

confidence: 99%

Factors associated with generalized pustular psoriasis progression among patients with psoriasis vulgaris in Japan: Results from a claims database study

Hayama,

Iwasaki,

Tian

et al. 2023

The Journal of Dermatology

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Of those patients diagnosed with generalized pustular psoriasis (GPP) in Japan, approximately 30% have a prior psoriasis vulgaris (PsV) diagnosis. Therefore, understanding factors associated with a GPP diagnosis is essential for early diagnosis of GPP in patients with PsV. This retrospective cohort study was conducted to identify associated factors for GPP diagnosis in patients with PsV. Eligible patients with two confirmed diagnoses of PsV with/without a confirmed GPP diagnosis (International Classification of Disease 10th revision codes L40.0 and L40.1, respectively) were identified from the Japanese Medical Data Center database (JMDC) (July 1, 2005–January 31, 2019). Weighted logistic regression was used to identify associated factors (based on recorded comorbidities) between the PsV only and PsV with GPP cohorts. Odds ratios (ORs) of ≥1.5, associated with a high probability of a GPP diagnosis, were reported for factors with ≥5 patients/cohort. The time from event to GPP diagnosis was evaluated. The highest associated factor for GPP diagnosis was psoriatic arthritis (OR 20.2, 95% confidence interval [CI] 17.06–23.92, P < 0.0001), which also had the shortest time from event to GPP diagnosis (median 119 days). Other comorbidities associated with GPP diagnosis were other psoriasis, tonsillitis, and sinusitis. Treatments associated with GPP diagnosis included systemic corticosteroids (OR 2.19, 95% CI 1.98–2.43, P < 0.0001; median time from treatment initiation to GPP diagnosis 180 days). Other associated treatments (other immunosuppressants, interleukin [IL]‐17 or IL‐23 inhibitors, and phototherapy) had a delay of ≥1 year from treatment initiation to GPP diagnosis. Back pain, headache, and fever were also identified as associated with a GPP diagnosis. Patients with PsV requiring systemic therapies are more likely to receive a GPP diagnosis than those not requiring systemic treatment. These data will help identify patients with PsV at high risk of developing GPP and potentially support early GPP diagnosis.

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Epidemiological survey of patients with pustular psoriasis in the Japanese Society for Psoriasis Research from 2017 to 2020

Cited by 10 publications

References 29 publications

A narrative review of the socioeconomic burden associated with generalised pustular psoriasis

A narrative review of the socioeconomic burden associated with generalised pustular psoriasis

Infantile generalized pustular psoriasis with excellent response to secukinumab: A case report

Factors associated with generalized pustular psoriasis progression among patients with psoriasis vulgaris in Japan: Results from a claims database study

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