{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T09:01:23Z","timestamp":1773910883739,"version":"3.50.1"},"reference-count":22,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T00:00:00Z","timestamp":1697587200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>IgG4\u2010related disease (IgG4\u2010RD) represents a recently characterized multisystemic fibroinflammatory condition that can manifest a spectrum of skin findings (IgG4\u2010related skin disease; IgG4\u2010RSD). Histopathologic and immunohistochemical criteria have been proposed; however, the specificity of these criteria merits scrutiny given the potential histopathologic overlap of IgG4\u2010RSD and both neoplastic and inflammatory skin conditions featuring lymphoplasmacytic infiltrates (IgG4\u2010RSD mimics). This study sought to assess the specificity of the criteria by quantifying the frequency by which an expanded spectrum of IgG4\u2010RSD mimics meet proposed thresholds.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Following IRB approval, a total of 69 cases of IgG4\u2010RD mimics, representing 14 different diagnoses featuring plasma cells, were reviewed and analyzed for the following histopathologic and immunohistochemical features: (i) maximum IgG4+ count\/high\u2010powered field (hpf) &gt;200; (ii) IgG4\/IgG ratio &gt;0.4 averaged over 3 hpfs; (iii) IgG4+ count &gt;10 per hpf.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Screening for IgG4\u2010RSD by histopathologic criteria demonstrated the high frequency of lymphoplasmacytic infiltrates, contrasted with the rarity of storiform fibrosis (only one case of erythema elevatum diutinum [EED]) and obliterative phlebitis (0 cases). By immunohistochemical criteria, the analysis revealed that no cases exceeded 200 IgG4+ cells; 13% (9\/69) cases demonstrated an IgG4\/IgG ratio of &gt;0.4 averaged over 3 hpfs; and 23% (16\/69) cases demonstrated a mean IgG4+ count of &gt;10 per hpf.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Application of proposed IgG4\u2010RSD histopathologic criteria to an expanded spectrum of potential IgG4\u2010RSD mimics (to include cutaneous marginal zone lymphoma, syphilis, necrobiosis lipoidica, lichen sclerosus, ALHE, psoriasis, lymphoplasmacytic plaque, EED, and erosive pustular dermatosis), highlights the relative nonspecificity of lymphoplasmacytic infiltrates contrasted with the stringency of storiform fibrosis and obliterative fibrosis. Furthermore, an IgG4+ cell count of &gt;10 per hpf and an IgG4\/IgG ratio of &gt;0.4 are not specific to IgG4\u2010RSD alone. In the appropriate clinical context for IgG4\u2010RSD, histopathologic features still represent the entry threshold for diagnosis consideration, which then allows for further screening by immunohistochemical criteria.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cup.14548","type":"journal-article","created":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T06:09:08Z","timestamp":1697695748000},"page":"163-169","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Assessment of specificity of dermatopathologic criteria for <scp>IgG4<\/scp>\u2010related skin disease"],"prefix":"10.1111","volume":"51","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5666-9999","authenticated-orcid":false,"given":"Zachary","family":"Skopec","sequence":"first","affiliation":[{"name":"Department of Dermatology The University of Iowa Hospitals and Clinics  Iowa City Iowa 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Iowa USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew","family":"Bellizzi","sequence":"additional","affiliation":[{"name":"Department of Pathology The University of Iowa Hospitals and Clinics  Iowa City Iowa USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1987-9521","authenticated-orcid":false,"given":"Vincent","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Dermatology The University of Iowa Hospitals and Clinics  Iowa City Iowa USA"},{"name":"Department of Pathology The University of Iowa Hospitals and Clinics  Iowa City Iowa 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