Pseudo-Ocular Cicatricial Pemphigoid : Bilateral Cicatrizing Conjunctivitis -- Chemical Ocular Burns In A Young Man - A Case Report

Cicatricial conjunctivitis is chronic disease with conjunctival fibrosis that may lead to alterations of conjunctival architecture, potentially sight-threatening. The medical history, physical exam, and laboratory tests often provide the diagnosis of the underlying disease. Causes of conjunctival cicatrization are not limited to autoimmune diseases, such as ocular cicatricial pemphigoid. Other well-known causes include thermal and chemical burns, Stevens-Johnson , Ocular rosacea, keratoconjunctivitis, eye drops in the treatment of glaucoma, often underdiagnosed.  This case study aims to describe a similar process, an atypical case about a Pseudo-ocular pemphigoid cicatricial after chemical ocular burns, limited only to the conjunctiva. 

We received a 35 yo man in the ophthalmological emergency department. The medical history was relevant for a chronic exposure to lime due to his mason profesion, the patient had nor associated ophtalmological or systemic symptoms or disease. He consults for a progressive visual acuity decline appearing since 6 months. He presented with chronic bilateral cicatrizing conjunctivitis.

On clinical examination, we had found in the left eye reduced vision : 20/400,with meibomian gland dysfunction, severe inflammation and eye dryness, upper tarsal scarring with lateral canthal fibrosis, partial limbal stem-cell deficiency, symblepharon formation and severe fornix shortening was also noted, with total ocular surface dermalization, in addition to deep corneal neovascularization.

In the right eye, visual acuity was 20/40,  we found  ocular surface dermalization with neovascularization only on the inferior half of the cornea.

No characteristic extraocular findings like peri-ocular vitiligo, alopecia,  and lack of sweating were found.

Nasofibroscopy wasnormal.

Conjunctival biopsy with immunostaining was performed, taken from the inferior nasal fornix, to distinguish for suspected mucus membrane pemphigoid : no vesiculobullous formation found.  The patient underwent ocular surface reconstruction with the aid of amniotic membrane.  After scar tissue was removed,  the first layer of the amniotic membrane was attached with tissue adhesive and fibrin glue while the second layer amniotic membrane, was placed over the cornea, the bulbar, and tarsal conjunctiva, and was secured with 8-0 Vicryl sutures to the conjunctival edges. Postoperative care : topical medications for inflammation alleviation. Systemic immunosuppressive agents were discussed, we indicated oral prednisolone. No recurrence of symblepharon was noted during the one year follow-up.

We aim at highlighting the important role of Amniotic membrane in the treatment of pseudo-ocular pemphigoid, or the benefit of cornea and ocular surface specialists, general ophthalmologists, and ophthalmology residents.

Further investigation is needed for providing evidence to incorporate the procedure into treatment protocol. A systematic approach is required for the clinical history, examination, and laboratory investigations of patients to arrive at the correct diagnosis of the underlying cause.  Effective management of patients requires knowledge of multiple modalities such as systemic immunosuppressive therapy, use of scleral contact lenses, and surgery for ocular surface and vision improvement

Conflict of interest : none declared