Siew Eng Choon, Peter Anthony Foley, Pravit Asawanonda, Hideki Fujita, Seong-Jin Jo, Yu-ling Shi, Colin Theng, Azura Mohd Affandi, Chul Hwan Bang, Maria Lorna Frez, Huang Yu Huei, Doanh Le Huu, Tae-Gyun Kim, Akimichi Morita, Hazel H. Oon, Pablo Fernández-Peñas, Natta Rajatanavin, Suganthy Robinson, Latha Selvarajah, Tsen-Fang Tsai
{"title":"Asia-Pacific consensus recommendations on the management of generalized pustular psoriasis","authors":"Siew Eng Choon, Peter Anthony Foley, Pravit Asawanonda, Hideki Fujita, Seong-Jin Jo, Yu-ling Shi, Colin Theng, Azura Mohd Affandi, Chul Hwan Bang, Maria Lorna Frez, Huang Yu Huei, Doanh Le Huu, Tae-Gyun Kim, Akimichi Morita, Hazel H. Oon, Pablo Fernández-Peñas, Natta Rajatanavin, Suganthy Robinson, Latha Selvarajah, Tsen-Fang Tsai","doi":"10.1111/1346-8138.17471","DOIUrl":"10.1111/1346-8138.17471","url":null,"abstract":"<p>Generalized pustular psoriasis (GPP) is a rare, chronic, heterogeneous, and potentially life-threatening disease characterized by primary, sterile, and macroscopically visible pustules with or without systemic symptoms. There are ethnic differences in the genetic mutations associated with GPP that might affect the clinical manifestations and treatment responses. Currently, there is limited evidence from the patient population in the Asia-Pacific (APAC) region, resulting in a general paucity of information on the effective management of patients with GPP in this region. This modified Delphi panel study aimed to identify current evidence and gain advanced insights to facilitate the development of a regionally tailored APAC consensus on the management of GPP. A systematic literature review (SLR) was conducted to identify published literature and develop consensus statements on (i) definition and clinical course, (ii) diagnosis of GPP, (iii) treatment outcomes, goals, and monitoring measures, and (iv) optimal management strategies and clinical practices. Statements were rated by a panel of dermatologists in two rounds, with the threshold for consensus at ≥80% agreement. Twenty experts from the APAC region reached consensus on 106 statements that were developed based on the SLR and experts' collective expertise. The experts agreed that GPP is a rare, severe, and potentially life-threatening condition that is distinct from plaque psoriasis. This consensus emphasized the importance of a tailored treatment strategy taking into account the GPP flare severity and each patient's unique clinical circumstances. The experts reached consensus on the severity classification of GPP flares and recommended first-line and maintenance treatment options for adult GPP, childhood GPP, and GPP in pregnancy. These consensus outcomes have been synthesized into treatment algorithms to guide dermatologists in the APAC region in their clinical decision-making processes.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"51 12","pages":"1579-1595"},"PeriodicalIF":2.9,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11624156/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142402548","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful treatment of Bothnian-type palmoplantar keratoderma using the etretinate and goreisan combination","authors":"Tomoko Oshimo, Ryoko Hamada, Chie Ono, Kazuyoshi Fukai, Eijiro Akasaka, Daisuke Tsuruta","doi":"10.1111/1346-8138.17394","DOIUrl":"10.1111/1346-8138.17394","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 1","pages":"e22-e23"},"PeriodicalIF":2.9,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142396482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of revertant mosaic-like normal-looking spots in a patient with erythroderma with IL36RN and CARD14 heterozygous mutations","authors":"Maho Matsuo, Xiaoyu Zang, Toshinari Miyauchi, Yoko Mizutani, Hirofumi Niwa, Kayoko Tanaka, Hiroaki Iwata","doi":"10.1111/1346-8138.17498","DOIUrl":"10.1111/1346-8138.17498","url":null,"abstract":"<p>An 89-year-old Japanese woman presented with erythroderma associated with significant scaling. A histological examination showed acanthosis with hyperkeratosis and hyperkeratinization of the hair follicles. Genetic analyses using DNA from the peripheral blood revealed heterozygous mutations in <i>IL36RN</i> (c.115+6T>C) and <i>CARD14</i> c.2648G>A (p.Arg883His). Based on these findings, we diagnosed her with erythroderma attributable to autoinflammatory keratinization disease. She then developed more than 30 small, round, well-defined, spots on her back and extremities that appeared histologically normal. We suspected that these spots might be revertant mosaicism. Immunohistochemical staining with p65, which is a component of nuclear factor kappa-light-chain-enhancer of activated B cells (NF-kB), revealed nuclear staining in epidermal keratinocytes in erythematous lesions, but not in the normal-looking spots. However, mutations in <i>IL36RN</i> and <i>CARD14</i> unexpectedly persisted in the epidermis and dermis of the normal-looking spots.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"51 12","pages":"1669-1673"},"PeriodicalIF":2.9,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11624151/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142383134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Detection of human papillomavirus in plantar warts and its impact on outcome","authors":"Akira Shimizu, Kosaka Mieko, Kayoko Yamaguchi, Osamu Niwa, Yasuhito Ishigaki, Masaru Sakurai","doi":"10.1111/1346-8138.17497","DOIUrl":"10.1111/1346-8138.17497","url":null,"abstract":"<p>Cutaneous warts are caused by human papillomavirus (HPV) infection. Distinguishing plantar warts from clavus and tylosis can be difficult. A less-invasive method of examining these lesions is necessary. Previously, we collected data on 90 patients with warts and related diseases to explore differentiation methods using HPV typing of tissue from the wart surface. In that study, 21 patients were diagnosed as cases with plantar warts, however, 10 of those 21 cases showed HPV-negative by polymerase chain reaction analysis, causing some ambiguity, thus their outcomes should be confirmed. To assess the role of HPV typing in clinical practice, we followed up these 21 cases (11 HPV-positive and 10 HPV-negative) and analyzed their outcomes. The HPV-positive group included HPV1a (one case), HPV27 (four cases), HPV57 (three cases), and HPV65 (three cases). The median age of the 21 patients was 43 years, that of the 11 HPV-positive cases was 37 years, and that of the 10 HPV-negative cases was 44 years. The sex ratios (male:female) of the HPV-positive and HPV-negative groups were 6:5 and 2:8, respectively. All 21 patients were treated with liquid nitrogen after surface keratin removal, concomitant with salicylic acid topical plaster or oral administration of Yokuinin. The longest follow-up period was 548 days. Kaplan–Meier analysis was performed to assess the healing rate according to HPV-positivity. The healing rate in HPV-positive cases was significantly higher than in HPV-negative cases (<i>P</i> = 0.001). Although the sample size was small, the results suggest HPV typing using non-invasive surface materials facilitates accurate diagnosis and prevents prolonged treatment of plantar warts.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 1","pages":"175-178"},"PeriodicalIF":2.9,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142383135","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Spesolimab, the first-in-class anti-IL-36R antibody: From bench to clinic","authors":"Akimichi Morita, Yukari Okubo, Shinichi Imafuku, Tadashi Terui","doi":"10.1111/1346-8138.17449","DOIUrl":"10.1111/1346-8138.17449","url":null,"abstract":"<p>Inflammatory diseases that are driven by several pro-inflammatory cytokines has resulted in in the development of targeted therapies across different disease settings. Interleukin (IL)-36 cytokines have been implicated in several inflammatory diseases. In this review we describe the scientific evidence surrounding the use of the IL-36 receptor (IL-36R)-targeting antibody, spesolimab, in IL-36-mediated skin diseases: generalized pustular psoriasis (GPP), palmoplantar pustulosis (PPP), hidradenitis suppurativa, and Netherton syndrome (NS). Spesolimab, a high affinity, specific, humanized, antagonistic immunoglobulin G1 antibody, targets the IL-36R at a binding site distinct from its agonists, IL-36α/β/γ, and at least one endogenous antagonist, IL-36R antagonist. In vitro and in vivo data for spesolimab show effective inhibition of IL-36R-mediated signaling pathways, and six Phase I studies in healthy volunteers presented a favorable safety and pharmacokinetic (PK) profile, leading to the development of a clinical trial program to evaluate spesolimab in the treatment of IL-36R-mediated diseases. Six studies (including an expanded access program) have evaluated the efficacy, safety, PKs, and pharmacogenomics of spesolimab in patients with GPP flares. Spesolimab treatment of GPP flares resulted in rapid and sustained improvements in pustular and skin clearance, and clinically significant improvements in patient-reported symptoms and quality of life. Spesolimab also significantly reduces the risk of GPP flares and flare occurrence, preventing disease worsening and has a favorable safety profile. There have been three trials of spesolimab in PPP; further evaluation is needed to better define those patients who might benefit from the treatment. A trial of spesolimab in NS is ongoing, while other spesolimab trials suggest that IL-36 may only play a secondary role in the pathogenesis of atopic dermatitis. In conclusion, research into spesolimab has provided much needed insight into the role of IL-36 in the human immune system and the mechanism behind IL-36-mediated inflammatory diseases. Spesolimab provides an efficacious targeted treatment for GPP, a disease with a high unmet medical need.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"51 11","pages":"1379-1391"},"PeriodicalIF":2.9,"publicationDate":"2024-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17449","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142383136","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of familial Mediterranean fever complicated by IgA vasculitis and psoriasis vulgaris","authors":"Takako Tsukamoto, Yohei Iwata, Naoki Ohmiya, Kazumitu Sugiura","doi":"10.1111/1346-8138.17470","DOIUrl":"10.1111/1346-8138.17470","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 2","pages":"e182-e183"},"PeriodicalIF":2.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142335574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nadine Wiedenmayer, Katharina Hogrefe, Silvia Mihalceanu, Julia K. Winkler, Alexander H. Enk
{"title":"Case report: A novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy","authors":"Nadine Wiedenmayer, Katharina Hogrefe, Silvia Mihalceanu, Julia K. Winkler, Alexander H. Enk","doi":"10.1111/1346-8138.17475","DOIUrl":"10.1111/1346-8138.17475","url":null,"abstract":"<p>Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis that is characterized by autoantibodies against epidermal adhesion proteins causing painful mucosal and skin blistering. Standard treatments for PV include corticosteroids, steroid-sparing immunosuppressants, or intravenous monoclonal anti–CD20-antibody therapy. The European guidelines suggest high-dose intravenous immunoglobulin (IVIg) therapy as a promising approach for severe or treatment-resistant cases. We report on a 65-year-old woman with severe and recurrent disease who achieved long-term disease stabilization with IVIg treatment. Because of recurrent fatigue and headache, the patient was switched to an alternative IVIg preparation with a novel manufacturing process, thus ensuring high purity and better tolerability. We observed excellent efficacy, yet side effects remained largely unchanged. Further studies are necessary to evaluate the long-term efficacy and tolerability of this new IVIg preparation.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"51 12","pages":"1665-1668"},"PeriodicalIF":2.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11624157/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142335581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Buruli ulcer: An epidemiological update from Japan","authors":"Ryo Fukaura, Manabu Ato, Chiaki Murase, Yuji Miyamoto, Mariko Sugawara-Mikami, Toshifumi Takahashi, Yoshihiko Hoshino, Noriki Fujimoto, Masashi Akiyama, Norihisa Ishii, Rie Yotsu","doi":"10.1111/1346-8138.17483","DOIUrl":"10.1111/1346-8138.17483","url":null,"abstract":"<p>Japan is one of the rare non-tropical countries with documented cases of Buruli ulcer (BU). <i>Mycobacterium ulcerans</i> subsp. <i>shinshuense</i> has been identified as the causative agent. The first report of BU in Japan dates back to 1982, with sporadic reports thereafter. Recently, the number of cases has been on the increase, and 50 cases (57.7%) are from the past decade alone, out of a total of 87 cases reported to date. Japan's well-developed healthcare facilities play a crucial role in enabling detailed investigations and providing appropriate treatment for patients, contributing to a favorable prognosis. However, the rarity of the disease results in lack of awareness among healthcare professionals, leading to frequent delays in diagnosis. This article aims to offer an updated overview of BU cases in Japan and to raise awareness of BU among dermatologists and other healthcare professionals in a non-endemic setting.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 1","pages":"3-10"},"PeriodicalIF":2.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17483","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142335580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}