{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T14:49:12Z","timestamp":1764859752150,"version":"build-2065373602"},"reference-count":33,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,7,20]],"date-time":"2025-07-20T00:00:00Z","timestamp":1752969600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reports"],"abstract":"<jats:p>Background and Clinical Significance: Ocular cicatricial pemphigoid (OCP) is a rare autoimmune disease affecting the conjunctiva and oral mucosa. Chronic inflammation causes conjunctival scarring, leading to symblepharon, trichiasis, corneal damage, and possible blindness. Diagnosis is clinical, supported by biopsy and immunofluorescence. Treatment includes systemic corticosteroids, immunosuppressants, and biologics in refractory cases. Case Presentation: A 64-year-old male presented with ocular irritation, trichiasis, and counting fingers (CF) visual acuity in the left eye. Slit-lamp examination revealed conjunctival inflammation, corneal epithelial defect, and symblepharon in the left eye. Biopsy confirmed ocular cicatricial pemphigoid (OCP). He was treated with topical steroids, cyclosporine, subconjunctival injections, and systemic corticosteroids, followed by surgery, which improved BCVA to 0.10 logMAR. Two years later, disease progression resulted in severe inflammation and visual decline in both eyes. Systemic azathioprine and corticosteroids achieved partial control. Due to insufficient response, rituximab therapy was initiated, leading to significant reduction in inflammation and stabilization of disease. Right eye BCVA improved to 0.16 logMAR; the left remained at CF. The patient continues to receive rituximab during exacerbations and is under regular follow-up. Conclusions: Early diagnosis and timely systemic treatment are essential in preventing vision loss in OCP. In refractory cases, biologic agents like rituximab may offer effective disease control.<\/jats:p>","DOI":"10.3390\/reports8030115","type":"journal-article","created":{"date-parts":[[2025,7,21]],"date-time":"2025-07-21T07:56:14Z","timestamp":1753084574000},"page":"115","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Rituximab Therapy in Refractory Ocular Cicatricial Pemphigoid: A Case Report"],"prefix":"10.3390","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8229-7826","authenticated-orcid":false,"given":"Sania Vidas","family":"Pauk","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6438-3034","authenticated-orcid":false,"given":"Antonela","family":"Geber","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-0526-8070","authenticated-orcid":false,"given":"Iva","family":"Be\u0161li\u0107","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5002-2588","authenticated-orcid":false,"given":"Ines","family":"Lako\u0161-Juki\u0107","sequence":"additional","affiliation":[{"name":"Department of Dermatovenereology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7137-5059","authenticated-orcid":false,"given":"Tomislav","family":"Kuzman","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, 10000 Zagreb, Croatia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"226","DOI":"10.22336\/rjo.2020.38","article-title":"Ocular cicatricial pemphigoid","volume":"64","author":"Stan","year":"2020","journal-title":"Rom. J. Ophthalmol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1097\/01.all.0000244799.33734.d4","article-title":"Ocular cicatricial pemphigoid","volume":"6","author":"Hingorani","year":"2006","journal-title":"Curr. Opin. Allergy Clin. Immunol."},{"key":"ref_3","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_4","first-page":"3379","article-title":"Ocular cicatricial pemphigoid (Review)","volume":"20","author":"Branisteanu","year":"2020","journal-title":"Exp. Ther. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1007\/s003470050021","article-title":"Okul\u00e4res vernarbendes Pemphigoid","volume":"97","author":"Messmer","year":"2000","journal-title":"Ophthalmologe"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1083\/jcb.142.1.271","article-title":"Hemidesmosome formation","volume":"142","author":"Schaapveld","year":"1998","journal-title":"J. Cell Biol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"270","DOI":"10.3109\/08820538.2011.588660","article-title":"Ocular cicatricial pemphigoid: A review","volume":"26","author":"Kirzhner","year":"2011","journal-title":"Semin. Ophthalmol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"79","DOI":"10.4274\/tjo.galenos.2022.34683","article-title":"Clinical approach to ocular cicatricial pemphigoid","volume":"53","author":"Korkmaz","year":"2023","journal-title":"Turk. J. Ophthalmol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1016\/j.ophtha.2020.07.052","article-title":"Autoantibody Detection for Diagnosis in Direct Immunofluorescence-Negative Mucous Membrane Pemphigoid: Ocular and Other Sites Compared","volume":"128","author":"Dart","year":"2021","journal-title":"Ophthalmology"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"2479","DOI":"10.1080\/09273948.2024.2413892","article-title":"Treatment of ocular surface disease in ocular cicatricial pemphigoid","volume":"32","author":"Abbott","year":"2024","journal-title":"Ocul. Immunol. Inflamm."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1440","DOI":"10.1097\/ICO.0000000000002683","article-title":"Rituximab as an adjuvant rescue treatment for ocular cicatricial pemphigoid","volume":"40","author":"Bevans","year":"2021","journal-title":"Cornea"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.autrev.2019.02.005","article-title":"Pemphigus and mucous membrane pemphigoid: An update","volume":"18","author":"Buonavoglia","year":"2019","journal-title":"Autoimmun. Rev."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1136\/bjo.79.11.1003","article-title":"Inferior retractor plication surgery for lower lid entropion with trichiasis","volume":"79","author":"Elder","year":"1995","journal-title":"Br. J. Ophthalmol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1111\/j.1442-9071.1993.tb00760.x","article-title":"Retractor plication for lower lid entropion","volume":"21","author":"Rosser","year":"1993","journal-title":"Aust. N. Z. J. Ophthalmol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e200","DOI":"10.1016\/j.jcjo.2023.03.009","article-title":"Long-term outcomes of fornix reconstruction and cicatricial entropion repair in ocular mucous membrane pemphigoid and drug-related secondary pemphigoid","volume":"59","author":"Khan","year":"2024","journal-title":"Can. J. Ophthalmol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1080\/08820530590931386","article-title":"The osteo-odonto-keratoprosthesis (OOKP)","volume":"20","author":"Liu","year":"2005","journal-title":"Semin. Ophthalmol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/S0161-6420(89)32942-3","article-title":"Glaucoma in patients with ocular cicatricial pemphigoid","volume":"96","author":"Tauber","year":"1989","journal-title":"Ophthalmology"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1007\/s00347-023-01863-z","article-title":"Differenzialdiagnosen des okul\u00e4ren Schleimhautpemphigoids [Differential diagnoses of ocular mucous membrane pemphigoid]","volume":"120","author":"Steven","year":"2023","journal-title":"Ophthalmologie"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.ajo.2017.08.009","article-title":"Clinical implications of direct immunofluorescence findings in patients with ocular mucous membrane pemphigoid","volume":"183","author":"Labowsky","year":"2017","journal-title":"Am. J. Ophthalmol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1080\/09273948.2020.1716988","article-title":"Reliability of Conjunctival Biopsy for Diagnosis of Ocular Mucous Membrane Pemphigoid: Redetermination of the Standard for Diagnosis and Outcomes of Previously Biopsy-Negative Patients","volume":"29","author":"Anesi","year":"2021","journal-title":"Ocul. Immunol. Inflamm."},{"key":"ref_21","unstructured":"American Academy of Ophthalmology (2014). Ophthalmic Pathology and Intraocular Tumors. Basic and Clinical Science Course (BCSC), American Academy of Ophthalmology."},{"key":"ref_22","unstructured":"American Academy of Ophthalmology (2014). External Disease and Cornea. Basic and Clinical Science Course (BCSC), American Academy of Ophthalmology."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1097\/ICO.0000000000003382","article-title":"Direct Immunofluorescence Findings and Factors Affecting Conjunctival Biopsy Positivity in Ocular Mucous Membrane Pemphigoid","volume":"43","author":"Jain","year":"2024","journal-title":"Cornea"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/S0039-6257(98)00005-8","article-title":"Management of conjunctival cicatrizing diseases and severe ocular surface dysfunction","volume":"43","author":"Chiou","year":"1998","journal-title":"Surv. Ophthalmol."},{"key":"ref_25","first-page":"1087","article-title":"Real-world experience of using ciclosporin-A 0.1% in the management of ocular surface inflammatory diseases","volume":"106","author":"Deshmukh","year":"2022","journal-title":"Br. J. Ophthalmol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e35","DOI":"10.1016\/j.oooo.2025.02.016","article-title":"Successful treatment of oral mucous membrane pemphigoid using tacrolimus and doxycycline","volume":"140","author":"Elamin","year":"2025","journal-title":"Oral Surg. Oral Med. Oral Pathol. Oral Radiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.jtos.2023.04.016","article-title":"Intralesional triamcinolone injection as an adjuvant therapy for refractory cicatrizing conjunctivitis","volume":"29","author":"Pivovar","year":"2023","journal-title":"Ocul. Surf."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1111\/exd.14829","article-title":"A systematic review on efficacy, safety and treatment durability of intravenous immunoglobulin in autoimmune bullous dermatoses: Special focus on indication and combination therapy","volume":"32","author":"Kianfar","year":"2023","journal-title":"Exp. Dermatol."},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Baffa, M.E., Corr\u00e0, A., Maglie, R., Mariotti, E.B., Montefusco, F., Pipit\u00f2, C., Senatore, S., Quintarelli, L., Caproni, M., and Antiga, E. (2022). Rituximab in Mucous Membrane Pemphigoid: A Monocentric Retrospective Study in 10 Patients with Severe\/Refractory Disease. J. Clin. Med., 11.","DOI":"10.3390\/jcm11144102"},{"key":"ref_30","first-page":"1221","article-title":"Rituximab in the treatment of ocular cicatricial pemphigoid: A retrospective cohort study. Graefes Arch","volume":"255","author":"You","year":"2017","journal-title":"Clin. Exp. Ophthalmol."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Bohelay, G., Alexandre, M., Le Roux-Villet, C., Sitbon, I., Doan, S., Soued, I., Shourick, J., Rousset, L., Mellottee, B., and Heller, M. (2022). Rituximab therapy for mucous membrane pemphigoid: A retrospective monocentric study with long-term follow-up in 109 patients. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.915205"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1016\/S0161-6420(91)32209-7","article-title":"Remission and recurrence after withdrawal of therapy for ocular cicatricial pemphigoid","volume":"98","author":"Neumann","year":"1991","journal-title":"Ophthalmology"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.ajo.2020.09.029","article-title":"Disease Relapse After Drug-Free Remission in Ocular Mucous Membrane Pemphigoid","volume":"223","author":"Shifera","year":"2021","journal-title":"Am. J. Ophthalmol."}],"container-title":["Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2571-841X\/8\/3\/115\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:12:57Z","timestamp":1760033577000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2571-841X\/8\/3\/115"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,20]]},"references-count":33,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,9]]}},"alternative-id":["reports8030115"],"URL":"https:\/\/doi.org\/10.3390\/reports8030115","relation":{},"ISSN":["2571-841X"],"issn-type":[{"type":"electronic","value":"2571-841X"}],"subject":[],"published":{"date-parts":[[2025,7,20]]}}}