{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T23:00:47Z","timestamp":1790290847986,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T00:00:00Z","timestamp":1790208000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T00:00:00Z","timestamp":1790208000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>IgG4\u2010related disease (IgG4\u2010RD) is a systemic fibroinflammatory disorder characterized by dense infiltration of IgG4\u2010positive plasma cells, fibrosis, and elevated serum IgG4 levels. Although various cutaneous manifestations of IgG4\u2010RD have been described, and vasculitic changes have occasionally been reported, granulomatous vasculitis is not considered a typical histopathological feature of cutaneous IgG4\u2010RD. We report a 93\u2010year\u2010old woman with a history of IgG4\u2010RD who presented with long\u2010standing subcutaneous nodules on both knees. Histopathological examination revealed dense lymphoplasmacytic infiltration with marked fibrosis, obliterative phlebitis, and granulomatous vasculitis characterized by vascular destruction with multinucleated giant cells. Immunohistochemistry demonstrated a marked increase in IgG4\u2010positive plasma cells with a high IgG4\/IgG\u2010positive plasma cell ratio. Comprehensive clinical, serological, and histopathological evaluation excluded other causes of granulomatous vasculitis, including eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, and sarcoid vasculitis. The lesions responded favorably to systemic corticosteroid therapy. To our knowledge, granulomatous vasculitis has not been recognized as a typical histopathological feature of cutaneous IgG4\u2010RD. This case suggests that granulomatous vasculitis may rarely occur in cutaneous lesions of IgG4\u2010RD, but additional cases are needed to confirm this association.<\/jats:p>","DOI":"10.1111\/cup.70217","type":"journal-article","created":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T22:56:06Z","timestamp":1790290566000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cutaneous\n                    <scp>IgG4<\/scp>\n                    \u2010Related Disease Accompanied by Granulomatous Vasculitis: A Case Report"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-4849-9383","authenticated-orcid":false,"given":"Tomoki","family":"Sakiyama","sequence":"first","affiliation":[{"name":"Department of Dermatology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3122-4528","authenticated-orcid":false,"given":"Toshiya","family":"Miyake","sequence":"additional","affiliation":[{"name":"Department of Dermatology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9332-0369","authenticated-orcid":false,"given":"Satoshi","family":"Nakamizo","sequence":"additional","affiliation":[{"name":"Department of Dermatology Kyoto University Graduate School of Medicine  Kyoto Japan"},{"name":"Alliance Laboratory for Advanced Medical Research, Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuto","family":"Fukutome","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masakazu","family":"Fujimoto","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Ehime University Graduate School of Medicine  Toon Ehime Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4322-9561","authenticated-orcid":false,"given":"Hironori","family":"Haga","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0773-0554","authenticated-orcid":false,"given":"Kenji","family":"Kabashima","sequence":"additional","affiliation":[{"name":"Department of Dermatology Kyoto University Graduate School of Medicine  Kyoto Japan"},{"name":"A*STAR Skin Research Labs (A*SRL), Skin Research Institute of Singapore (SRIS), and Singapore Immunology Network (SIgN), Agency for Science, Technology, and Research (A*STAR)  Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,24]]},"reference":[{"issue":"9","key":"e_1_2_9_2_1","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1038\/modpathol.2012.72","article-title":"Consensus Statement on the Pathology of IgG4\u2010Related Disease","volume":"25","author":"Deshpande V.","year":"2012","journal-title":"Modern Pathology"},{"issue":"3","key":"e_1_2_9_3_1","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1080\/14397595.2020.1859710","article-title":"The 2020 Revised Comprehensive Diagnostic (RCD) Criteria for IgG4\u2010RD","volume":"31","author":"Umehara H.","year":"2021","journal-title":"Modern Rheumatology"},{"issue":"5","key":"e_1_2_9_4_1","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1111\/bjd.13296","article-title":"IgG4\u2010Related Skin Disease","volume":"171","author":"Tokura Y.","year":"2014","journal-title":"British Journal of Dermatology"},{"issue":"1","key":"e_1_2_9_5_1","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jaad.2016.01.046","article-title":"Cutaneous Manifestations of IgG4\u2010Related Disease (RD): A Systematic Review","volume":"75","author":"Charrow A.","year":"2016","journal-title":"Journal of the American Academy of Dermatology"},{"issue":"3","key":"e_1_2_9_6_1","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/1346-8138.13723","article-title":"Immunoglobulin G4\u2010Related Disease and Its Skin Manifestations","volume":"44","author":"Takayama R.","year":"2017","journal-title":"Journal of Dermatology"},{"issue":"10","key":"e_1_2_9_7_1","doi-asserted-by":"crossref","first-page":"2738","DOI":"10.1111\/all.16686","article-title":"IgG4\u2010Related Disease: Emerging Roles of Novel Genetic Variants, Immune Cell Subsets and Therapeutic Targets","volume":"80","author":"Tedesco L.","year":"2025","journal-title":"Allergy"},{"issue":"4","key":"e_1_2_9_8_1","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1111\/his.12658","article-title":"Lymph Node Granulomas in Immunoglobulin G4\u2010Related Disease","volume":"67","author":"Bateman A. C.","year":"2015","journal-title":"Histopathology"},{"issue":"4","key":"e_1_2_9_9_1","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1007\/s12308-011-0117-5","article-title":"Perifollicular Granulomatous Inflammation in Reactive Lymph Nodes: A Possible Morphologic Marker for IgG4 Plasmacytosis","volume":"4","author":"Siddiqi I. N.","year":"2011","journal-title":"Journal of Hematopathology"},{"issue":"3","key":"e_1_2_9_10_1","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1038\/modpathol.2011.177","article-title":"Histopathological Findings in 29 Lymph Node Biopsies With Increased IgG4 Plasma Cells","volume":"25","author":"Grimm K. E.","year":"2012","journal-title":"Modern Pathology"},{"issue":"4","key":"e_1_2_9_11_1","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1007\/s12026-022-09279-8","article-title":"ANCA\u2010Associated Vasculitis and IgG4\u2010Related Disease Overlap Syndrome: A Case Report and Literature Review","volume":"70","author":"Faz\u2010Mu\u00f1oz D.","year":"2022","journal-title":"Immunology Research"},{"issue":"10","key":"e_1_2_9_12_1","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1016\/j.autrev.2017.07.020","article-title":"Antineutrophil Cytoplasmic Antibody\u2010Associated Vasculitides and IgG4\u2010Related Disease: A New Overlap Syndrome","volume":"16","author":"Danlos F. X.","year":"2017","journal-title":"Autoimmunity Reviews"},{"issue":"10","key":"e_1_2_9_13_1","doi-asserted-by":"crossref","first-page":"1926","DOI":"10.1111\/1756-185X.13693","article-title":"Do ANCA\u2010Associated Vasculitides and IgG4\u2010Related Disease Really Overlap or Not?","volume":"22","author":"Erden A.","year":"2019","journal-title":"International Journal of Rheumatic Diseases"},{"issue":"4","key":"e_1_2_9_14_1","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1097\/DAD.0000000000003197","article-title":"IgG4\u2010Related Cutaneous Vasculitis Presenting as Multiple Subcutaneous Nodules","volume":"48","author":"Olson G. S.","year":"2026","journal-title":"American Journal of Dermatopathology"},{"issue":"5","key":"e_1_2_9_15_1","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2023.1145257","article-title":"Eosinophilic Granulomatosis With Polyangiitis \u2013 Advances in Pathogenesis, Diagnosis, and Treatment","volume":"10","author":"Fijolek J.","year":"2023","journal-title":"Frontiers in Medicine"},{"issue":"11","key":"e_1_2_9_16_1","article-title":"Eosinophilic Granulomatosis With Polyangiitis: An Overview","volume":"5","author":"Gioffredi A.","year":"2014","journal-title":"Frontiers in Immunology"},{"issue":"8","key":"e_1_2_9_17_1","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2024.1369233","article-title":"Granulomatosis With Polyangiitis: Clinical Characteristics and Updates in Diagnosis","volume":"11","author":"Potentas\u2010Policewicz M.","year":"2024","journal-title":"Frontiers in Medicine"},{"issue":"12","key":"e_1_2_9_18_1","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1111\/ijd.16247","article-title":"Cutaneous Manifestations of Antineutrophil Cytoplasmic Antibody\u2010Associated Vasculitis ( AAV ): A Concise Review With Emphasis on Clinical and Histopathologic Correlation","volume":"61","author":"Gibson L. E.","year":"2022","journal-title":"International Journal of Dermatology"},{"issue":"1","key":"e_1_2_9_19_1","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/j.ccm.2023.08.004","article-title":"Cutaneous Sarcoidosis","volume":"45","author":"Abdelghaffar M.","year":"2024","journal-title":"Clinics in Chest Medicine"},{"issue":"10","key":"e_1_2_9_20_1","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1111\/1346-8138.13364","article-title":"Case of Giant Cell Arteritis as a Manifestation of Immunoglobulin G4\u2010Related Disease","volume":"43","author":"Takaki M.","year":"2016","journal-title":"Journal of Dermatology"},{"issue":"6","key":"e_1_2_9_21_1","doi-asserted-by":"crossref","first-page":"623","DOI":"10.3899\/jrheum.2024-0861","article-title":"Concurrent IgG4\u2010Related Disease and Giant Cell Arteritis: A Rare Case Study","volume":"52","author":"Gener K. R.","year":"2025","journal-title":"Journal of Rheumatology"}],"container-title":["Journal of Cutaneous Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cup.70217","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/cup.70217","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cup.70217","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T22:56:09Z","timestamp":1790290569000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/cup.70217"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,24]]},"references-count":20,"alternative-id":["10.1111\/cup.70217"],"URL":"https:\/\/doi.org\/10.1111\/cup.70217","archive":["Portico"],"relation":{},"ISSN":["0303-6987","1600-0560"],"issn-type":[{"value":"0303-6987","type":"print"},{"value":"1600-0560","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,24]]},"assertion":[{"value":"2026-05-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-15","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-24","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"cup.70217"}}