Ocular cicatricial pemphigoid review

Curr Opin Allergy Clin Immunol. 2004 Oct;4(5):435-9. doi: 10.1097/00130832-200410000-00017.


Purpose of review: Ocular mucous membrane pemphigoid produces progressive cicatrizing conjunctivitis; this can result in scarring of the conjunctiva and cornea.

Recent findings: Increased expression of macrophage-colony-stimulating factor, collagen-binding heat shock protein 47, transforming growth factor-beta1, IL-4, IL-5, and macrophage migration inhibitory factor may enhance both conjunctival inflammation and conjunctival scarring. Recent developments on mucous membrane pemphigoid medical therapy include the efficacious effect of daclizumab, intravenous immunoglobulin therapy, and methotrexate. Subconjunctival mitomycin has proved not to be efficacious in controlling long-term, conjunctival inflammation and scarring. The Boston scleral lens enhances vision, reduces the disabling ocular pain and photophobia, and helps to heal persistent epithelial defects, reducing recurrence of defects. Recent developments on mucous membrane pemphigoid surgical therapy include keratolimbal allografts and amniotic membrane transplantation, with or without penetrating keratoplasty for ocular surface reconstruction in total stem cell deficiency. The prognosis is strongly influenced by preoperative conditions such as tear function and functional external ocular adnexae, and by postoperative conditions such as persistent inflammation, severe dry eye, or rejection of the keratolimbal allograft. Some authors find efficacious the use of amniotic membrane transplantation for reconstruction of the conjunctival fornices provided systemic immunosuppression is pre- and post-operatively used.

Summary: The use of daclizumab or intravenous immunoglobulin therapy, the better selection of candidates for surgical interventions, and the better pre- and post-operative management of keratolimbal allograft and amniotic membrane transplantation may improve visual rehabilitation in the patients with ocular mucous membrane pemphigoid.

Publication types

  • Review

MeSH terms

  • Conjunctival Diseases / diagnosis
  • Conjunctival Diseases / immunology*
  • Conjunctival Diseases / therapy
  • Humans
  • Pemphigoid, Benign Mucous Membrane / diagnosis
  • Pemphigoid, Benign Mucous Membrane / immunology*
  • Pemphigoid, Benign Mucous Membrane / therapy