When Standard Treatments Are Not Enough: Rituximab Offers Hope for Refractory Ocular Cicatricial Pemphigoid

Ocular cicatricial pemphigoid, sometimes called ocular mucous membrane pemphigoid, is a rare autoimmune condition that attacks the conjunctiva, the thin membrane covering the white of the eye. Left untreated or inadequately controlled, it causes progressive scarring that can fuse the eyelid to the eyeball, turn the eyelashes inward to scratch and damage the cornea, and ultimately result in blindness. It is notoriously difficult to manage, and even patients on treatment can continue to deteriorate. A case report published in the journal Reports in July 2025 describes a patient who failed multiple treatment strategies before rituximab finally brought his disease under control.

The condition begins as chronic immune-mediated inflammation of the conjunctiva, driven by autoantibodies that attack proteins in the basement membrane zone of the mucous membrane. Over time, the inflammation produces fibrosis and scarring. Clinically, this appears as symblepharon (adhesions between the eyelid and the eye surface), trichiasis (inward-pointing eyelashes), corneal damage, and in advanced cases, complete opacification and loss of useful vision. Diagnosis requires a biopsy showing the characteristic pattern of immune deposits along the basement membrane, confirmed by direct immunofluorescence.

The patient described in this report was a 64-year-old man who presented with ocular irritation, trichiasis, and visual acuity in his left eye that had deteriorated to counting fingers at close range. Slit-lamp examination revealed conjunctival inflammation, a corneal epithelial defect, and symblepharon in the left eye. After biopsy confirmed the diagnosis, he was started on topical steroids, topical cyclosporine, subconjunctival steroid injections, and systemic corticosteroids. Surgical intervention followed and brought his left eye vision to 0.10 logMAR on the logarithm of the minimum angle of resolution scale.

Two years later, the disease flared severely. Inflammation returned in both eyes and vision in both deteriorated. Systemic azathioprine was added alongside continued corticosteroids, achieving only partial disease control. Because the response was insufficient, the clinical team initiated rituximab therapy. Rituximab works by targeting and depleting CD20-positive B cells, disrupting the autoimmune cycle that drives this condition. After rituximab, the patient experienced a significant reduction in inflammation and stabilization of the disease. His right eye improved to 0.16 logMAR, while his left eye, where pre-existing damage was more extensive, remained at counting fingers acuity. He continues to receive rituximab infusions when exacerbations occur and remains under close follow-up.

The case joins a small but growing body of reports supporting rituximab and other biologics as options for refractory ocular cicatricial pemphigoid. Because the disease is rare and clinical trials are limited, much of the current evidence base comes from case series and case reports. The authors of this report emphasize a key message that runs through this literature: early and accurate diagnosis, combined with timely escalation to systemic treatment, is the most important factor in protecting long-term vision. Waiting too long to escalate therapy allows irreversible scarring to accumulate. For patients whose disease cannot be controlled with conventional immunosuppression, agents like rituximab represent a meaningful step forward.