Rituximab Therapy in Refractory Ocular Cicatricial Pemphigoid: A Case Report

<b>Background and Clinical Significance</b>: 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. <b>Case Presentation</b>: 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. <b>Conclusions</b>: 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.

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Publication Details

Journal
Reports — Medical Cases Images and Videos
Published
2025-07-20
DOI
https://doi.org/10.3390/reports8030115
Citations
1
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
Field-Weighted Citation Impact
1.47
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article

Rituximab Therapy in Refractory Ocular Cicatricial Pemphigoid: A Case Report

Tomislav Kuzman, Ines Lakoš-Jukić, Sania Vidas Pauk, Antonela Geber et al.
1 citations
Reports — Medical Cases Images and Videos
Autoimmune Bullous Skin Diseases
1.47
article

Rituximab Therapy in Refractory Ocular Cicatricial Pemphigoid: A Case Report

Tomislav Kuzman, Ines Lakoš-Jukić, Sania Vidas Pauk, Antonela Geber, Iva Bešlić
article en
1 citations

Abstract

<b>Background and Clinical Significance</b>: 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. <b>Case Presentation</b>: 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. <b>Conclusions</b>: 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.

Reports — Medical Cases Images and VideosVol. 8(3)
University Hospital Centre Zagreb (HR), University of Zagreb (HR)
Good health and well-being
Openalex Percentile: Top 17%
Autoimmune Bullous Skin Diseases
1.47
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