{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T22:42:53Z","timestamp":1789684973323,"version":"4.0.1"},"reference-count":12,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,3,20]],"date-time":"2023-03-20T00:00:00Z","timestamp":1679270400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Immunoglobulin G4-related disease (IgG4-RD) is a rare fibro-inflammatory condition characterized by IgG4-expressing plasma cell infiltration of the skin and other organs, leading to profound itchiness. Oral corticosteroids are the first-line therapy for IgG4-RD but relapses and potential side effects are common. In this case, we discuss a patient with a hyperpigmented, scaling dermatitis on his arms, back, and chest with lichen amyloidosis (LA) that incompletely responded to corticosteroids. He had reduced quality of life secondary to chronic pruritus. Dupilumab, an IL-4 and IL-13 inhibitor, was initiated. He experienced a transient worsening, followed by complete resolution of his itch with remission of his rash. While the pathogenesis of IgG4-RD is not entirely understood, a T-helper 2 (Th2) immune response has been implicated, with interleukins (IL) 4, 5, 10, and 13 playing a role in IgG4 class switch, resulting in eosinophilia and elevated IgE. The strong response of dupilumab in this case may provide evidence in favor of the involvement of IL-4 and IL-13 in the pathogenesis of cutaneous IgG4-RD. Future clinical studies involving larger patient populations may be warranted.<\/jats:p>","DOI":"10.3390\/life13030833","type":"journal-article","created":{"date-parts":[[2023,3,20]],"date-time":"2023-03-20T06:48:35Z","timestamp":1679294915000},"page":"833","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Immunoglobulin G4-Related Disease-Associated Dermatitis with Pruritus: A Positive Response to Dupilumab"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1717-8943","authenticated-orcid":false,"given":"Tyler","family":"Beck","sequence":"first","affiliation":[{"name":"Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC 29425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Plante","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC 29425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"India","family":"Robinson","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC 29425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katsiaryna","family":"Khatskevich","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, SC 29425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Forcucci","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC 29425, USA"},{"name":"Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, SC 29425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Valdebran","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, SC 29425, USA"},{"name":"Department of Pediatrics, Medical University of South Carolina, Charleston, SC 29425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,20]]},"reference":[{"key":"ref_1","first-page":"e97","article-title":"Amyloid A amyloidosis secondary to immunoglobulin G4\u2013related disease","volume":"60","year":"2020","journal-title":"Rheumatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/1346-8138.13723","article-title":"Immunoglobulin G4-related disease and its skin manifestations","volume":"44","author":"Takayama","year":"2017","journal-title":"J. Dermatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jaad.2016.01.046","article-title":"Cutaneous manifestations of IgG4-related disease (RD): A systematic review","volume":"75","author":"Charrow","year":"2016","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.5070\/D3256044441","article-title":"Cutaneous and systemic IgG4-related disease: A review for dermatologists","volume":"25","author":"Shenoy","year":"2019","journal-title":"Dermatol. Online J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1111\/ijd.13369","article-title":"IgG4-related skin disease may have distinct systemic manifestations: A systematic review","volume":"55","author":"Bennett","year":"2016","journal-title":"Int. J. Dermatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"10262","DOI":"10.1038\/s41598-018-28405-x","article-title":"Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: A multicentre study","volume":"8","author":"Shirakashi","year":"2018","journal-title":"Sci. Rep."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1007\/s40257-017-0278-9","article-title":"Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review","volume":"18","author":"Weidner","year":"2017","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_8","first-page":"A4727","article-title":"Primary localised cutaneous amyloidosis--a systematic review","volume":"60","author":"Kaltoft","year":"2013","journal-title":"Dan. Med. 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J."},{"key":"ref_12","first-page":"269","article-title":"Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease","volume":"69","author":"Mattoo","year":"2013","journal-title":"Allergy"}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/13\/3\/833\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:58:59Z","timestamp":1760122739000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/13\/3\/833"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,20]]},"references-count":12,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,3]]}},"alternative-id":["life13030833"],"URL":"https:\/\/doi.org\/10.3390\/life13030833","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,20]]}}}