{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T04:50:40Z","timestamp":1791867040782,"version":"4.3.4"},"reference-count":10,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T00:00:00Z","timestamp":1675296000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dermatopathology"],"abstract":"<jats:p>Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and nonspecific presentation. We present the case of a 69-year-old female in which MMP of the vulva was not initially suspected. The first biopsy, from lesional tissue for routine histology, revealed fibrosis, late-stage granulation tissue, and nonspecific findings. A second biopsy, from perilesional tissue for direct immunofluorescence (DIF), revealed DIF findings typical of MMP. Scrutiny of both the first and second biopsies revealed a subtle but telling histologic feature: subepithelial clefts along adnexae in the context of a scarring process with neutrophils and eosinophils, which can be an important clue to MMP. This histologic clue has been previously described; reinforcing its importance may prove useful for future cases, especially those for which DIF is not feasible. Our case demonstrates the protean presentations of MMP, the need for persistence in sampling unusual cases, and the relevance of inconspicuous histologic features. The report highlights this underrecognized yet potentially decisive histologic clue to MMP, reviews current biopsy guidelines when MMP is suspected, and delineates the clinical and morphological features of vulvar MMP.<\/jats:p>","DOI":"10.3390\/dermatopathology10010009","type":"journal-article","created":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T04:55:12Z","timestamp":1675313712000},"page":"63-69","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines"],"prefix":"10.3390","volume":"10","author":[{"given":"Jason R.","family":"McFadden","sequence":"first","affiliation":[{"name":"Department of Biological Sciences, Dartmouth College, Hanover, NH 03755, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5750-1605","authenticated-orcid":false,"given":"Advaita S.","family":"Chaudhari","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, Dartmouth College, Hanover, NH 03755, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debra","family":"Birenbaum","sequence":"additional","affiliation":[{"name":"Department of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA"},{"name":"Department of Obstetrics & Gynecology, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynette","family":"Margesson","sequence":"additional","affiliation":[{"name":"Department of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA"},{"name":"Department of Obstetrics & Gynecology, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Gonzalez","sequence":"additional","affiliation":[{"name":"Department of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aravindhan","family":"Sriharan","sequence":"additional","affiliation":[{"name":"Department of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA"},{"name":"Department of Pathology & Laboratory Medicine, Geisel School of Medicine at Dartmouth, Hanover, NH 03755, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,2,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1016\/j.cden.2013.07.003","article-title":"Mucous membrane pemphigoid","volume":"57","author":"Xu","year":"2013","journal-title":"Dent. Clin. N. Am."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1016\/0190-9622(91)70159-Y","article-title":"Cicatricial pemphigoid","volume":"24","author":"Ahmed","year":"1991","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1111\/j.1346-8138.2009.00793.x","article-title":"From anti-p200 pemphigoid to anti-laminin \u03b31 pemphigoid","volume":"37","author":"Dainichi","year":"2010","journal-title":"J. Dermatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1111\/j.1365-2133.2009.09508.x","article-title":"Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid: IgA and IgE autoantibodies in antilaminin-332 MMP","volume":"162","author":"Natsuga","year":"2010","journal-title":"Br. J. 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[2nd ed.]."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4103\/0253-7184.93786","article-title":"Vesicobullous disorders of female genitalia","volume":"33","author":"Garg","year":"2012","journal-title":"Indian J. Sex. Transm. Dis. AIDS"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1926","DOI":"10.1111\/jdv.17395","article-title":"European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology\u2013Part II","volume":"35","author":"Schmidt","year":"2021","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1016\/j.jaad.2017.05.016","article-title":"Multiple and repeated sampling increases the sensitivity of direct immunofluorescence testing for the diagnosis of mucous membrane pemphigoid","volume":"77","author":"Shimanovich","year":"2017","journal-title":"J. Am. Acad. Dermatol."}],"container-title":["Dermatopathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2296-3529\/10\/1\/9\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:22:10Z","timestamp":1760120530000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2296-3529\/10\/1\/9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,2]]},"references-count":10,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,3]]}},"alternative-id":["dermatopathology10010009"],"URL":"https:\/\/doi.org\/10.3390\/dermatopathology10010009","relation":{},"ISSN":["2296-3529"],"issn-type":[{"value":"2296-3529","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,2]]}}}