Surgical Management In A Case Of Ocular Surface Inflammation Secondary To Pemphigus Foliaceus

Pemphigus Foliaceus (PF) is an acquired, autoimmune disorder marked by circulating immunoglobulin G autoantibodies that target desmoglein-1, causing the formation of blisters in the skin epidermis. Ocular symptoms are infrequent, with symptoms conjunctival hyperaemia and irritation. Corneal melt is a sight-threatening complication, occasionally seen in autoimmune blistering disorders. This describes the progressive thinning of the corneal stroma, which can lead to further complications including corneal perforation. We describe a case of ocular surface inflammation secondary to PF with corneal melting, secondary infection and subsequent perforation. We outline the surgical and systemic management of this rare complication.

A 72-year-old male presented to the emergency department of a large tertiary hospital in London with an acute exacerbation of PF. He was admitted under the dermatology team and commenced on mycophenolate mofetil, oral prednisolone and intravenous teicoplanin for secondary cellulitis. Soon after admission, he was referred to ophthalmology due to ocular symptoms of bilateral redness, discharge, blurred vision and crusting around the eyelids. 

BCVA was 6/24 bilaterally. Examination showed bilateral conjunctival injection with no cicatrisation. The right cornea had a subtotal epithelial defect with inferior corneal melt (~80%). The left cornea had a small epithelial defect <1mm in the inferior mid-periphery with associated 20% melt as well as peripheral melt with no associated epithelial defect. (Photos available) Empiric treatment included topical Moxifloxacin, topical Ganciclovir, and oral Aciclovir.

Over subsequent reviews, the right eye progressed to corneal melt and perforation measuring approximately 3.0mm. Following unsuccessful attempts at corneal gluing, an emergency tectonic patch graft was performed, with topical and oral steroids post-operatively to suppress inflammation. Unfortunately, the corneal melt continued to progress with superotemporal graft dehiscence and iris show (see figures)

A second 10.0mm tectonic patch graft was performed, with three days of intravenous Methylpredisolone prior to surgery to further suppress ocular surface inflammation. An amniotic membrane overlay was sutured over the graft and a bandage contact lens placed. Rituximab was commenced for long-term immunosuppression.

On his last review, the vision in the right eye was PL and vision in the left eye was stable. The left eye while quiescent with signs of previous corneal melt, due to the prior ocular surface inflammation, showed filamentary keratitis, for which topical acetylcysteine and ciclosporin was commenced.

PF and other autoimmune bullous diseases can cause ocular surface inflammation. When poorly controlled this may lead to corneal melt and perforation, a severe and sight-threatening complication with devasting impacts.

Further studies understanding of the mechanisms behind ocular surface involvement in PF are required, allowing development of treatment pathways. This case highlights the necessity for prompt referral and assessment of ocular symptoms in acute dermatological conditions such as PF as well as the importance of a multi-disciplinary team approach and collaboration between dermatologists and ophthalmologists to diagnose and treat such disorders. We demonstrate a surgical management plan including the post-operative complications.