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Monocular corneal blindness due to ocular cicatricial pemphigoid: case presentation

Poster Details

First Author: M.Radenkovic SERBIA

Co Author(s):    P. Jovanovic   G. Stankovic Babic   M. Trenkic Bozinovic   M. Zivkovic   S. Markovic   V. Lazarevic

Abstract Details

Purpose:

Ocular cicatricial pemphigoid (OCP) is chronic, progressive, systemic autoimmune disease of mucous membrane (oral mucosa, conjunctiva, pharynx, larynx, trachea, esophagus,genital mucosa), afecting skin rarely, characterized by T-lymphocyte dysregulation and production of circulating autoantibodies directed against variety of adhesion molecules in the hemidesmosome-epithelial membrane complex. In geneticaly susceptible individuals with HLA-DR2, HLA-DR4 and HLA-DQw7 triggering agent cause autoantibodies production against α,β subunits of integrin and α,β,γ subunits of laminin5. Hallmark feature:chronic cicatrizing conjunctivitis, progressive conjunctival fibrosis and corneal epitheliopathy. Based on clinical findings, disease is divided into 4 stages.

Setting:

Examination and diagnose was perform at Eye Clinic Nis based on clinical presentation. Stage I: chronic conjunctivitis and subepithelial tarsal conjunctival fibrosis.Stage II: conjunctival cicatrization, fornix foreshortening. Stage III: symblepharon, aberrant lash growth, cicatricial entropion. Stage IV: dry eye, ankyloblepharon, corneal keratinization, could be completely vascularized and keratinized.

Methods:

D.V (50,female) in February 2013. referred to ophthalmologist due to pain and redness of left eye caused by corneal epithelial and stromal defect.Visual acuity after healing VOD=1,0 VOS=0,4; TOU=13mmHg. After couple of months administering artefitial drops and persistant dryness of eyes patient referred to cornea specialist. Slit lamp right eye: reduced precorneal lacrimal film, subconjunctival fibrosis and scarring , whitish appearance. Slit lamp left eye: central corneal opacification, superfitialy and profoundly vascularized, keratinization of left eye, fornix foreshortening, symblepharon. Right eye fundus: normal finding. Blood test normal, ANA and rheumatoid factor negative, holesterol 8,39; HDL 1,5; LDL 5,96.

Results:

After systemic examination only nasal involvement was detected as erosions of nasal vestibule and septum. Topical(Cyclosporine A) and systemic therapy(Metotrexate 7,5mg weekly, Prednisolon) was administered. Due to intolerance therapy was discontinued. Six months later topical and peroral Doxyciclin 50mg for 2 weeks was prescribed with transient effect. Due to actual local finding: Ocular cicatricial pemphigoid gradus II/III; VOD=0,5; VOS=light perception, local therapy(artefitial drops and ointment, NSAIL)and systemic prednisolon 1mg/kg was continued. Indirect imunofluorescence and Elisa test was not perform due to technical impossibility. These is case of clinically but not biopsy-proven progressive cicatricial pemphigoid affecting eyes.

Conclusions:

Left untreated, OCP results in severe conjunctival scarring and sight-threatening manifestations. Main intention is conjunctival inflammation control, fibrosis prevention, ocular discomfort and visual acuity improvement, reduction in extraocular mucosal lesions. Local/systemic immunomodulatory therapy is the treatment of choice for controlling disease activity and progression. Low-dose weekly methotrexate is first-line treatment in mild-to-moderate disease as adjunctive high-dose prednisolone. Immunoglobulin therapy 2-3g/kg is safe and effective for treatment-resistant pemphigoid. Ocular surface reconstruction requires allograft limbal or amniotic membrane transplantation, tarsorrhaphy, followed by serum-derived tears. Corneal scarring requires penetrating or lamellar keratoplasty even keratoprosthesis. Treatment requires multidisciplinary approach for optimal management. FINANCIAL DISCLOUSRE: NONE

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