{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T07:18:03Z","timestamp":1782544683259,"version":"3.54.5"},"reference-count":7,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T00:00:00Z","timestamp":1759363200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.mdpi.com"],"crossmark-restriction":true},"short-container-title":["Dermatopathology"],"abstract":"<jats:p>Human herpesvirus-8 (HHV-8)-associated multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder with systemic and cutaneous manifestations that can be diagnostically challenging, especially in immunocompromised patients. We report the case of a 68-year-old man with HIV and biopsy-proven Kaposi sarcoma (KS), who developed progressive fevers, night sweats, weight loss, and fatigue, accompanied by diffuse lymphadenopathy, splenomegaly, and new erythematous and hyperpigmented lesions shortly after intravenous immunoglobulin therapy for Guillain\u2013Barr\u00e9 syndrome. A laboratory evaluation revealed that the patient had elevated total protein and polyclonal hypergammaglobulinemia, without monoclonality. Imaging demonstrated widespread lymphadenopathy and splenomegaly. A core lymph node biopsy showed polytypic plasmacytosis, but was non-diagnostic. Given the ongoing symptoms, an excisional biopsy was performed, revealing regressed germinal centers with increased interfollicular vascularity, mantle zone \u201conion skinning,\u201d and HHV-8 LANA-1 nuclear positivity, establishing the diagnosis of HHV-8-associated MCD. Rituximab monotherapy was initiated, resulting in clinical improvement, resolution of the constitutional symptoms, and stabilization of ascites. This case highlights the importance of maintaining a high index of suspicion for MCD in patients with KS who develop new systemic or cutaneous findings, the limitations of a core biopsy, and the value of a timely excisional biopsy in guiding diagnosis and treatment.<\/jats:p>","DOI":"10.3390\/dermatopathology12040033","type":"journal-article","created":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T08:20:28Z","timestamp":1759393228000},"page":"33","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Diagnostic Challenges in HHV-8-Associated Multicentric Castleman Disease in a Patient with Prior Kaposi Sarcoma"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-6214-3348","authenticated-orcid":false,"given":"Seraphima S.","family":"Sidhom","sequence":"first","affiliation":[{"name":"Michigan State University College of Human Medicine, Grand Rapids, MI 49503, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-3838-916X","authenticated-orcid":false,"given":"Luke A.","family":"Laconi","sequence":"additional","affiliation":[{"name":"Michigan State University College of Human Medicine, Grand Rapids, MI 49503, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher A.","family":"LaFond","sequence":"additional","affiliation":[{"name":"Michigan State University College of Human Medicine, Grand Rapids, MI 49503, USA"},{"name":"Department of General Surgery, Munson Medical Center, Traverse City, MI 49684, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven C.","family":"Weindorf","sequence":"additional","affiliation":[{"name":"Department of General Surgery, Munson Medical Center, Traverse City, MI 49684, USA"},{"name":"Department of Pathology, Munson Medical Center, Traverse City, MI 49684, USA"},{"name":"Grand Traverse Pathology, PLLC, Traverse City, MI 49684, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,10,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1646","DOI":"10.1182\/blood-2016-10-746933","article-title":"International, evidence-based consensus diagnostic criteria for HHV-8\u2013associated multicentric Castleman disease","volume":"129","author":"Fajgenbaum","year":"2017","journal-title":"Blood"},{"key":"ref_2","first-page":"720","article-title":"Clinical presentation and survival of HIV-associated multicentric Castleman\u2019s disease","volume":"153","author":"Hoffmann","year":"2011","journal-title":"Br. J. Haematol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1086\/654798","article-title":"An interleukin-6\u2013related systemic inflammatory syndrome in patients co-infected with Kaposi sarcoma\u2013associated herpesvirus and HIV but without multicentric Castleman disease","volume":"51","author":"Uldrick","year":"2010","journal-title":"Clin. Infect. Dis."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Polizzotto, M.N., Uldrick, T.S., Hu, D., and Yarchoan, R. (2012). Clinical manifestations of Kaposi sarcoma herpesvirus lytic activation: Multicentric Castleman disease (KSHV\u2013MCD) and the KSHV inflammatory cytokine syndrome. Front. Microbiol., 3.","DOI":"10.3389\/fmicb.2012.00073"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3350","DOI":"10.1200\/JCO.2007.10.6732","article-title":"Prospective study of rituximab in chemotherapy-dependent human immunodeficiency virus associated multicentric Castleman\u2019s disease: ANRS 117 CastlemaB Trial","volume":"25","author":"Galicier","year":"2007","journal-title":"J. Clin. Oncol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1111\/bjh.15019","article-title":"The full spectrum of Castleman disease: 273 patients studied over 20 years","volume":"180","author":"Oksenhendler","year":"2018","journal-title":"Br. J. Haematol."},{"key":"ref_7","first-page":"202","article-title":"HHV-8 Linked to Kaposi\u2019s Sarcoma and Castleman\u2019s Disease in HIV-1-infected Patient: Case Report and Review of the Literature","volume":"18","author":"Zaghdoudi","year":"2024","journal-title":"Int. J. Hematol. Oncol. Stem Cell Res."}],"container-title":["Dermatopathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2296-3529\/12\/4\/33\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T08:59:32Z","timestamp":1759395572000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2296-3529\/12\/4\/33"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,2]]},"references-count":7,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["dermatopathology12040033"],"URL":"https:\/\/doi.org\/10.3390\/dermatopathology12040033","relation":{},"ISSN":["2296-3529"],"issn-type":[{"value":"2296-3529","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,2]]}}}