Posters

Blinding ocular cicatricial pemphigoid: one case report

Poster Details

First Author: F.El Alami MOROCCO

Co Author(s): Z. Chajai   A. Mchachi   L. Benhmidoune   A. Chakib   R. Rachid   M. Elbelhadji     

Abstract Details

Purpose:

Ocular cicatricial pemphigoid is a form of mucous membrane pemphigoid that features a chronic, relapsing-remitting bilateral conjunctivitis. Incidence rates vary between one in 12,000 to one in 60,000.Ultimately, patients affected by this autoimmune disease will experience conjunctival cicatrization or scarring, and if unresponsive to treatment or left untreated will develop corneal opacification and permanent vision loss.MMP can also affect the skin as well as mucous membranes of the mouth, nose, esophagus, genitals,and anus causing erosions, blisters, and strictures.Treatment is multidisciplinary and directed towards providing symptomatic relief and to stop the underlying destructive autoimmune process which is essential to prevent blindness.

Setting:

Adult Ophthalmology department, 20 August Hospital, Ibn Rochd University Hospital , Faculty of medicine and pharmacy, Hassan II University, Casablanca. Morocco

Methods:

We report on severe ocular involvement in a 56-year-old male, ophthalmologic findings included conjunctival cicatrization and symblepharon, corneal ulceration and corneal perforation, despite immunosuppressive therapy with cyclophosphamide and corticosteroids.

Results:

A 56- year-old male, followed for cicatricial ocular pemphigoid since 1 year with corticosteroids and cyclophosphamides, presented with complaints  of redness, watering and decreased visual acuity in both  eyes . On examination, vision was  hand motion in both eyes . On slit lamp evaluation of the right eye there was a thickened lid margin, conjunctival  congestion and subepithelial fibrosis , inferior symblepharon, cornea  showed  diffuse  punctate epitheliopathy, nasal neovascularisation, and central corneal perforated ulcer . for the left eye, the evaluation shows conjunctival congestion and subepithelial  fibrosis, ankyloblepharon, central corneal scare with neovascularisation and diffuse  punctate epitheliopathy.

Conclusions:

ocular cicatricial pemphigoid can be a diagnostic and therapeutic challenge since it can present with different symptoms in different stages of the disease. Early diagnosis and treatment are essential to achieving  permanent  remission.  Delay  in  diagnosis  and  therapy  leads  to  progression  and  eventually  blindness.  Current  research  has  identified  on  the  gene  that predisposes  to  ocular cicatricial pemphigoid  and  the target  antigen.  This  will  help  develop  newer  diagnostic  and therapeutic strategies.

Financial Disclosure:

None