Gastrointestinal bleeding with severe mucosal involvement in a patient with generalized pustular psoriasis without <i><scp>IL</scp>36<scp>RN</scp></i> mutation

Komatsuda, Shiori; Kamata, Masahiro; Chijiwa, Chika; Namiki, Kaoru; Fukaya, Saki; Hayashi, Kotaro; Fukuyasu, Atsuko; Tanaka, Takamitsu; Ishikawa, Takeko; Ohnishi, Takamitsu; Abe, Koichiro; Yamamoto, Takatsugu; Aozasa, Naohiko; Sugiura, Kazumitsu; Tada, Yayoi

doi:10.1111/1346-8138.14711

Cited by 7 publications

(5 citation statements)

References 9 publications

(13 reference statements)

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“…Komatsuda et al, 44 reported a case of severe mucosal involvement of the gastrointestinal lesions leading to gastrointestinal bleeding in a GPP patient. Oesophagogastroduodenoscopy (OGD) done for the patient showed three erosions on the oesophagus and a duodenal ulcer with visible vessel.…”

Section: Resultsmentioning

confidence: 99%

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%

Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Lim

Oon

2023

Experimental Dermatology

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“…DITRA is characterized by sudden eruptions of sterile widespread pustules across the body (9). Although GPP is sometimes accompanied by gastrointestinal complications (71), there are no reports of patients with DITRA developing intestinal pathology, and the relevancy of IL-36RN mutation to mucosal involvement remains to be elucidated. Although the IL-36R axis is relatively well studied in the context of skin inflammation using IL-36R KO and gain-of-function approaches, the emerging reports on the role of IL-36 in the gut are mainly confined to IL-36R deficiency (30,31,37), which may introduce potential shortcomings associated with gene targeting approaches.…”

Section: Discussionmentioning

confidence: 99%

Enhanced IL-36R signaling promotes barrier impairment and inflammation in skin and intestine

et al. 2020

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Deficiency in interleukin-36R (IL-36R) antagonist caused by loss-of-function mutations in IL-36RN leads to DITRA (deficiency of IL-36 receptor antagonist), a rare inflammatory human disease that belongs to a subgroup of generalized pustular psoriasis (GPP). We report a functional genetic mouse model of DITRA with enhanced IL-36R signaling analogous to that observed in patients with DITRA, which provides new insight into our understanding of the IL-36 family of molecules in regulating barrier integrity across multiple tissues. Humanized DITRA-like mice displayed increased skin inflammation in a preclinical model of psoriasis, and in vivo blockade of IL-36R pathway using anti-human IL-36R antibody ameliorated imiquimod-induced skin pathology as both prophylactic and therapeutic treatments. Deeper characterization of the humanized DITRA-like mice revealed that deregulated IL-36R signaling promoted tissue pathology during intestinal injury and led to impairment in mucosal restoration in the repair phase of chronic dextran sulfate sodium (DSS)–induced colitis. Blockade of IL-36R pathway significantly ameliorated DSS-induced intestinal inflammation and rescued the inability of DITRA-like mice to recover from mucosal damage in vivo. Our results indicate a central role for IL-36 in regulating proinflammatory responses in the skin and epithelial barrier function in the intestine, suggesting a new therapeutic potential for targeting the IL-36R axis in psoriasis and at the later stages of intestinal pathology in inflammatory bowel disease.

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“…35 Severe mucosal involvement with pharyngeal and gastrointestinal lesions leading to gastrointestinal bleeding has been described in single cases with generalized pustular psoriasis. 36 Perianal psoriasis is common in genital areas, but involvement of anal canal is not yet observed. 37 Psoriasis patients have a higher risk of liver abnormalities than the general population, including non-alcoholic fatty liver disease, drug-induced hepatitis, alcoholic hepatitis and neutrophilic cholangitis.…”

Section: Gi Involvement In Psoriasismentioning

confidence: 99%

“…Oral psoriasis without skin or nail stigmata has been described but was questioned 35 . Severe mucosal involvement with pharyngeal and gastrointestinal lesions leading to gastrointestinal bleeding has been described in single cases with generalized pustular psoriasis 36 . Perianal psoriasis is common in genital areas, but involvement of anal canal is not yet observed 37 .…”

Section: Gi Involvement In Psoriasismentioning

confidence: 99%

Gastrointestinal involvement of primary skin diseases

Hsieh

Wei

et al. 2020

Acad Dermatol Venereol

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Less is known about gastrointestinal (GI) involvement of primary skin diseases due to the difference in embryology, histology, microbiology and physiology between integument and alimentary tract. Oesophagus, following the oropharyngeal mucosa, is the most common GI segment affected by primary skin diseases, especially by eosinophilic oesophagitis, lichen planus and autoimmune bullous dermatoses like pemphigus vulgaris, mucosal membrane pemphigoid and epidermolysis bullosa acquisita. Eosinophilic oesophagitis is an emerging chronic atopic disease with oesophageal dysfunction as the typical presentation, and oesophageal narrowing, rings and stricture as late complications. Oesophageal lichen planus mainly involves the proximal to mid-oesophagus in elderly aged women with long-term oral mucosal lesions. In acute attack of pemphigus vulgaris, oesophageal involvement is not uncommon but often neglected and may cause sloughing oesophagitis (oesophagitis dissecans superficialis) with acute GI bleeding in rare cases. GI manifestation of hereditary bradykininergic angio-oedema with colicky acute abdomen mostly affects small intestine, usually in the absence of pruritus or urticaria, and is more severe and long-lasting than the acquired histaminergic form. Strong evidence supports association between inflammatory bowel disease, especially Crohn disease, and hidradenitis suppurativa/acne inversa. Patients with vitiligo need surveillance of autoimmune liver disease, autoimmune atrophic gastritis or coeliac disease when corresponding symptoms become suspect. Melanoma is the most common primary tumour metastatic to the GI tract, with small intestine predominantly targeted. Gastrointestinal involvement is not uncommon in disseminated mycosis fungoides. Extramammary Paget's disease is an intraepidermal adenocarcinoma of controversial origin, and a high association between the anogenital occurrence and colorectal adenocarcinoma has been reported. As GI tract is the largest organ system with multidimensional functions, dermatologists in daily practice should be aware of the gastrointestinal morbidities related to primary skin diseases for an early diagnosis and treatment.

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Gastrointestinal bleeding with severe mucosal involvement in a patient with generalized pustular psoriasis without IL36RN mutation

Cited by 7 publications

References 9 publications

Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Gastrointestinal and hepatic manifestations in patients with generalised pustular psoriasis

Enhanced IL-36R signaling promotes barrier impairment and inflammation in skin and intestine

Gastrointestinal involvement of primary skin diseases

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