Generalized pustular psoriasis from heterozygous <i>IL36RN</i> mutation developed after liver surgery

Fukushima, Hidehiko; Iwata, Y.; Numata, Shigeki; Saito, Kenta; Watanabe, Soichiro; Kobayashi, Tatsuhiko; Sugiura, Kazumitsu

doi:10.1111/1346-8138.14828

Cited by 1 publication

(3 citation statements)

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“…IL36RN mutation (deficiency of interleukin [IL]‐36 receptor antagonist [DITRA]) is found in recent years to be associated with GPP 26 . In 2019, a case of GPP in a patient with heterozygous IL36RN mutations, was reported after the patient underwent surgery for hepatocellular carcinoma 27 . It is postulated that IL36RN is involved in cancer development and may be associated with hepatocellular carcinoma.…”

Section: Resultsmentioning

confidence: 99%

“…A single intravenous infusion of infliximab 300 mg (5 mg/kg body weight) was effective therapy for a von Zumbusch pustular flare with neutrophilic cholangitis, presenting with transaminitis of ALT 682 U/L (normal range 14–54), AST 309 U/L (15–41), ALP 347 U/L (38–126). There was normalisation of the liver enzymes day 6 post‐infliximab administration 20 .…”

Section: Resultsmentioning

confidence: 99%

“…26 In 2019, a case of GPP in a patient with heterozygous IL36RN mutations, was reported after the patient underwent surgery for hepatocellular carcinoma. 27 It is postulated that IL36RN is involved in cancer development and may be associated with hepatocellular carcinoma. The case suggested that the liver injury from the surgery for hepatocellular carcinoma was a precipitant for GPP with DITRA.…”

Section: Hepatocellular Carcinoma Il36rn Mutation (Deficiency Of Inte...mentioning

confidence: 99%

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Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Lim

Oon

2023

Experimental Dermatology

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Generalised pustular psoriasis is a rare and severe form of pustular psoriasis. It was first described by Leopold von Zumbusch in 1910. 1 In 2017, The European Rare and Severe Psoriasis Expert Network (ERASPEN) classified pustular psoriasis into three phenotypes, namely generalised pustular psoriasis (GPP), acrodermatitis continua of Hallopeau and palmoplantar pustulosis. 2 GPP is defined by persisting or relapsing macroscopically visible sterile primary pustules occurring on non-acral skin and not within psoriasis plaques. The Japanese guidelines in 2018 diagnose GPP based on four primary parameters: (a) systemic symptoms such as fever and fatigue; (b) systemic or extensive flush accompanied by multiple sterile pustules that sometimes merge to form lakes of pus; (c) neutrophilic subcorneal pustules histopathologically characterised by Kogoj's spongiform pustules; and (d) recurrence of the described clinical and histological features. 3 A definite diagnosis of GPP is made with the presence of all four criteria.The prevalence of GPP is higher in Asians (7.46 per million in Japan) 4 compared to the Caucasian population (1.76 per million in France 5 and 3.8 per 100 000 in Sweden). 6 There is a female preponderance for GPP and the mean age at onset of GPP was 40.9 years. 7

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Hepatocellular Carcinoma Il36rn Mutation (Deficiency Of Inte...mentioning

confidence: 99%

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Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Lim

Oon

2023

Experimental Dermatology

View full text Add to dashboard Cite

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Generalized pustular psoriasis from heterozygous IL36RN mutation developed after liver surgery

Cited by 1 publication

References 5 publications

Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

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