You are here

Article Text

Article menu
Images In...
Turning a blind eye: ocular mucous membrane pemphigoid
Free
  1. Suran Loshana Fernando1,
  2. Phillip Myers2
  1. 1
    Royal North Shore Hospital, Clinical Immunology and Allergy, Reserve Road, St. Leonards, New South Wales, 2065, Australia
  2. 2
    Bankstown Hospital, Ophthalmology, Eldridge Road, Bankstown, New South Wales, 2200, Australia
  1. Suran Loshana Fernando, sfernando{at}nsccahs.health.nsw.gov.au

https://doi.org/10.1136/bcr.02.2009.1584

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    A man in his 60s presented with dry eyes and progressive deterioration in vision bilaterally over a 2-year period. He was treated a number of times presumptively for conjunctivitis with topical antibiotics. Upon referral to an ophthalmologist, physical examination revealed the presence of conjunctival scarring with loss of the fornix, symblepharon (adhesion between conjunctival surfaces of the lid and globe), entropion (lid inversion due to conjunctival scarring) and trichiasis (aberrant lashes causing abrasion of the cornea) (figs 1–3). The conjuctival signs were readily apparent when the patient was instructed to gaze laterally and vertically. There was no clinical involvement of other mucosal or skin surfaces. A conjuctival biopsy revealed subepithelial separation at the level of the basement membrane with stromal infiltration of lymphocytes and dense fibrous tissue. Direct immunofluoresence demonstrated the characteristic linear deposits of IgG along the basement membrane (fig 4). Local therapies that were implemented included lubricants, punctual occlusion and destruction of aberrant lash follicles. He also required systemic immunosuppressive therapy with oral prednisone, initially given at 0.5 mg/kg/day. His visual acuity then gradually improved and after 2 years is maintained at a dose of 0.1 mg/kg/day.

    Figure 1
    Figure 1

    Lid deformities with ectropion (lid eversion) of the upper lid and entropion (lid inversion) of the lower lid.

    Figure 2
    Figure 2

    Scarring of the conjunctival surface with trichiasis (aberrant lashes) resulting in corneal abrasion.

    Figure 3
    Figure 3

    Symblepharon formation (adhesion of the conjuctival surfaces of lid and globe) resulting in narrowing of the formix.

    Figure 4
    Figure 4

    Direct immunofluorescence of a conjunctival biopsy demonstrating linear deposition of IgG along the basement membrane zone.

    The diagnosis of ocular mucous membrane pemphigoid is often missed or delayed and chronic changes can lead to reduction in vision from corneal scarring and ulceration and dry eyes from lacrimal duct occlusion.1 Early diagnosis through recognition of ocular cicatrisation supported by the demonstration of autoimmune-mediated damage to the basement membrane by direct immunofluoresence enables local and systemic therapies to be more effective in ameliorating long term complications.2,3

    REFERENCES

    1. ↵
      1. Shiu M,
      2. McNabb AA
      . Cicatricial entropion and trichiasis in an urban Australian population. Clin Exp Ophthalmol 2005; 33: 582–5.
      OpenUrlCrossRefPubMedWeb of Science
    2. ↵
      1. Saw VP,
      2. Dart JK
      . Ocular mucous membrane pemphigoid: diagnosis and management strategies. Ocul Surf 2008; 6: 128–42.
      OpenUrlPubMedWeb of Science
    3. ↵
      1. Thorne JE,
      2. Woreta FA,
      3. Jabs DA,
      4. et al
      . Treatment of ocular mucous membrane pemphigoid with immunosuppressive drug therapy. Ophthalmology 2008; 115: 2146–52.
      OpenUrlCrossRefPubMedWeb of Science

    Footnotes

    • Competing interests: None.

    • Patient consent: Patient/guardian consent was obtained for publication.