{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T11:20:05Z","timestamp":1787743205512,"version":"build-2784847793"},"reference-count":11,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,8,18]]},"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:p>A 60-year-old man presented with severe, treatment-refractory bullous pemphigoid and was subsequently diagnosed with stage IV pleomorphic variant mantle cell lymphoma (MCL). Direct immunofluorescence and histopathology confirmed bullous pemphigoid, while peripheral blood smear abnormalities and cross-sectional imaging prompted further workup. Lymph node biopsy demonstrated a monotypic CD20+, PAX5+, CD5+, cyclin D1+B\u2009cell lymphoproliferative disorder, with CCND1::IGH fusion confirmed by fluorescence in situ hybridisation. The temporal association between cutaneous disease onset and lymphoma diagnosis, together with improvement of bullous pemphigoid following MCL-directed therapy, supports a paraneoplastic relationship. Bullous pemphigoid is most commonly idiopathic but is recognised in association with haematological malignancies. Its occurrence with MCL is exceedingly rare, with only two prior cases in the literature. This case reinforces the importance of screening for occult malignancy in patients with atypical, severe or refractory bullous disease.<\/jats:p>","DOI":"10.1136\/bcr-2026-274014","type":"journal-article","created":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T10:35:44Z","timestamp":1787740544000},"page":"e274014","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Mantle cell lymphoma presenting as bullous pemphigoid: a rare paraneoplastic syndrome"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5017-8884","authenticated-orcid":false,"given":"Avi","family":"Harisingani","sequence":"first","affiliation":[{"name":"Internal Medicine, MacNeal Hospital, Berwyn, Illinois, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nishant","family":"Tiwari","sequence":"additional","affiliation":[{"name":"Hematology and Medical Oncology, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taylor","family":"Zak","sequence":"additional","affiliation":[{"name":"Pathology, Loyola University Medical Center, Maywood, Illinois, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikita","family":"Gupta","sequence":"additional","affiliation":[{"name":"Internal Medicine, SUNY Upstate Medical University, Syracuse, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Priyanka","family":"Dudani","sequence":"additional","affiliation":[{"name":"Internal Medicine, MacNeal Hospital, Berwyn, Illinois, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Hagen","sequence":"additional","affiliation":[{"name":"Hematology and Medical Oncology, Loyola University Medical Center, Maywood, Illinois, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,8,26]]},"reference":[{"key":"2026082603350951000_19.8.e274014.1","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1111\/j.1365-2133.1981.tb00883.x","article-title":"Bullous pemphigoid: the frequency of mucosal involvement and concurrent malignancy related to indirect immunofluorescence findings","volume":"105","author":"Hodge","year":"1981","journal-title":"Br J Dermatol"},{"key":"2026082603350951000_19.8.e274014.2","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1975.01630200051003"},{"key":"2026082603350951000_19.8.e274014.3","doi-asserted-by":"crossref","DOI":"10.1001\/archderm.1978.01640180094035","article-title":"Coexistence of malignancies with bullous pemphigoid","volume":"114","author":"Chorzelski","year":"1978","journal-title":"Arch Dermatol"},{"key":"2026082603350951000_19.8.e274014.4","doi-asserted-by":"crossref","first-page":"1496","DOI":"10.1001\/archderm.140.12.1496","article-title":"Bullous pemphigoid associated with mantle cell lymphoma","volume":"140","author":"Iranzo","year":"2004","journal-title":"Arch Dermatol"},{"key":"2026082603350951000_19.8.e274014.5","doi-asserted-by":"publisher","DOI":"10.1016\/0020-7292(91)90751-P"},{"key":"2026082603350951000_19.8.e274014.6","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(74)90179-X"},{"key":"2026082603350951000_19.8.e274014.7","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.1980.00330220077028"},{"key":"2026082603350951000_19.8.e274014.8","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1999.02940.x"},{"key":"2026082603350951000_19.8.e274014.9","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2000.03674.x"},{"key":"2026082603350951000_19.8.e274014.10","unstructured":"WHO Classification of Tumours Editorial Board . 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