Cicatricial changes in ocular pemphigus

Eye (Lond). 2014 Apr;28(4):459-65. doi: 10.1038/eye.2014.3. Epub 2014 Jan 31.

Abstract

Purpose: To describe the clinical characteristics of ocular involvement in patients with pemphigus at an ophthalmological referral center.

Methods: A retrospective review was conducted on patients with the immunopathological diagnosis of pemphigus examined between 1 January 2000 and 1 April 2010. Uncorrected distance visual acuity (UDVA), best corrected distance visual acuity (BCVA), ocular symptoms, and ocular surface inflammatory and scarring changes were assessed.

Results: A total of 15 patients were identified, with a mean age of 68.27 ± 14.35 years, and 80% (n=12) were female. Extraocular involvement was reported in one patient. All of the eyes showed cicatricial changes in the conjunctiva. In all, 6 eyes (20%) were classified as stage I; 12 eyes (40%) as stage II; 10 eyes (33%) as stage III; and 2 eyes (7%) as stage IV. A statistically significant association was found between BCVA and the severity of ocular involvement. The mean BCVA logMAR was 1.66 (20/914), with a range from logMAR 0 (20/20) to logMAR 4 (NLP). Other ocular diseases were found in 8 (53.3%), systemic diseases in 10 (66.7%), and the use of pemphigus-inducing drugs in 10 patients (66.7%).

Conclusions: The present report represents the largest series of ocular involvement in pemphigus confirmed by immunopathology. The clinical manifestations varied from conjunctival hyperemia to corneal scarring and perforation. There was a strong association between scarring changes and low BCVA. Ocular and systemic diseases as well as the use of pemphigus-inducing drugs may predispose to ocular cicatricial changes observed in this series.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Cicatrix / pathology*
  • Conjunctival Diseases / drug therapy
  • Conjunctival Diseases / etiology
  • Conjunctival Diseases / pathology*
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Pemphigus / complications
  • Pemphigus / drug therapy
  • Pemphigus / pathology*
  • Retrospective Studies
  • Severity of Illness Index
  • Visual Acuity

Substances

  • Immunosuppressive Agents