{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:15:15Z","timestamp":1787026515162,"version":"build-2736575974"},"reference-count":15,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,5,26]],"date-time":"2022-05-26T00:00:00Z","timestamp":1653523200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Hidradenitis Suppurativa (HS) is a chronic suppurative disease of the pilosebaceous unit. The current model of HS pathophysiology describes the condition as the product of hyperkeratinisation and inflammation at the hair follicular unit. Environmental factors (such as smoking and obesity), gender, genetic predisposition, and skin dysbiosis are considered the main pathogenic drivers of the disease. Autoinflammatory syndromes associated with HS are rare but may help to highlight the potential roles of autoinflammation and dysregulated innate immune system in HS. Therefore, it is of major relevance to increase the awareness about these diseases in order to improve the understanding of the disease and to optimize the management of the patients. Herein, we report for the first time, to our knowledge, two clinical cases of Hyper-IgD syndrome-associated HS. Hyper-IgD is an autoinflammatory syndrome caused by a mevalonate kinase deficiency (MKD), a key kinase in the sterol and isoprenoid production pathway. We describe the potentially shared pathophysiological mechanisms underpinning comorbid MKD-HS and propose therapeutic options for the management of these patients.<\/jats:p>","DOI":"10.3389\/fimmu.2022.883811","type":"journal-article","created":{"date-parts":[[2022,6,3]],"date-time":"2022-06-03T06:47:12Z","timestamp":1654238832000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":14,"title":["Case Report: Comorbid Hyper-IgD Syndrome and Hidradenitis Suppurativa \u2013 A New Syndromic Form of HS? A Report of Two Cases"],"prefix":"10.3389","volume":"13","author":[{"given":"Philippe","family":"Guillem","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dillon","family":"Mintoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariam","family":"Kabbani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elie","family":"Cogan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginie","family":"Vlaeminck-Guillem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnes","family":"Duquesne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farida","family":"Benhadou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,5,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2021.1677","article-title":"Prevalence of Hidradenitis Suppurativa: A Systematic Review and Meta-Regression Analysis","volume":"157","author":"Jfri","year":"2021","journal-title":"JAMA Dermatol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1111\/exd.14214","article-title":"What Causes Hidradenitis Suppurativa ?-15 Years After","volume":"29","author":"Zouboulis","year":"2020","journal-title":"Exp Dermatol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.17982","article-title":"Inter-Rater and Intrarater Agreement and Reliability in Clinical Staging of Hidradenitis Suppurativa\/Acne Inversa","volume":"181","author":"Zouboulis","year":"2019","journal-title":"Br J Dermatol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.17716","article-title":"Inter-Rater Agreement and Reliability of Outcome Measurement Instruments and Staging Systems Used in Hidradenitis Suppurativa","volume":"181","author":"Thorlacius","year":"2019","journal-title":"Br J Dermatol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.15748","article-title":"Development and Validation of the International Hidradenitis Suppurativa Severity Score System (IHS4), a Novel Dynamic Scoring System to Assess HS Severity","volume":"177","author":"Zouboulis","year":"2017","journal-title":"Br J Dermatol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2020.00068","article-title":"Hidradenitis Suppurativa: Current Understanding of Pathogenic Mechanisms and Suggestion for Treatment Algorithm","volume":"7","author":"Seyed Jafari","year":"2020","journal-title":"Front Med (Lausanne)"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1930","DOI":"10.12688\/f1000research.17267.1","article-title":"A Systematic Review and Critical Evaluation of Inflammatory Cytokine Associations in Hidradenitis Suppurativa","volume":"7","author":"Frew","year":"2018","journal-title":"F1000Res"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1159\/000509873","article-title":"Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? 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