{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T01:42:15Z","timestamp":1791337335200,"version":"4.3.1"},"reference-count":26,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,2,9]],"date-time":"2020-02-09T00:00:00Z","timestamp":1581206400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The Japanese guidelines for the management of pemphigus (<jats:styled-content style=\"fixed-case\">JG<\/jats:styled-content>) were published in 2010. However, further progress in the treatment of pemphigus requires their validation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To examine the efficacy and safety of treatments based on the <jats:styled-content style=\"fixed-case\">JG<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective study of 84 Japanese patients with moderate to severe pemphigus, who were initially treated in accordance with the <jats:styled-content style=\"fixed-case\">JG<\/jats:styled-content> and then followed up for &gt;2\u00a0years, was performed in a single centre. Treatment typically consisted of 0.5\u20131\u00a0mg prednisone (<jats:styled-content style=\"fixed-case\">PSL<\/jats:styled-content>)\/kg\/day accompanied by 100\u00a0mg azathioprine\/day as a steroid\u2010sparing agent.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In 83 of the 84 patients (98.8%), complete remission on minimal therapy (\u226410\u00a0mg <jats:styled-content style=\"fixed-case\">PSL<\/jats:styled-content>\/day and concomitant immunosuppressive agent) was achieved. The time between initiation of therapy and remission was 13.9\u00a0\u00b1\u00a09.4\u00a0months. In 78 patients (92.9%), remission was accomplished within the 2\u2010year follow\u2010up. The 32 patients with recalcitrant disease (38.1%) received additional treatment. Relapse occurred in 12 patients (14.3%) either during tapering of the <jats:styled-content style=\"fixed-case\">PSL<\/jats:styled-content> dose (six patients) or after achieving remission (six patients). Adverse events, mostly liver enzyme elevation, infections and diabetes, occurred in 67 patients (79.8%). One patient (1.2%) died during the observation period after gastrointestinal haemorrhage.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our results suggested that the elderly and patients requiring additional therapies were at higher risk of adverse events, including severe infections, and should thus be monitored carefully. This study provided clinical data that could inform revised guidelines and contribute to the evaluation of future novel therapies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.16188","type":"journal-article","created":{"date-parts":[[2020,1,10]],"date-time":"2020-01-10T22:51:44Z","timestamp":1578696704000},"page":"1324-1330","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Results of the guideline\u2010based treatment for pemphigus: a single\u2010centre experience with 84 cases"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0581-4703","authenticated-orcid":false,"given":"R.","family":"Kakuta","sequence":"first","affiliation":[{"name":"Department of Dermatology Keio University School of Medicine Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.","family":"Kurihara","sequence":"additional","affiliation":[{"name":"Department of Dermatology Keio 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