Ocular Cicatricial Pemphigoid

Change Language German


  • Autoimmune process, allergic hypersensitivity reaction Type II

Clinical Presentation

  • Age typically 60-80 years, more common in women (female-to-male ratio 2:1)
  • Symptoms: insidiously starting or recurrent nonspecific bilateral conjunctivitis, often initially misdiagnosed as dry eyes


  • Conjunctivitis, diffuse hyperemia
  • Subconjunctival fibrosis
  • Fornix shortening
  • Keratinization of the caruncle/absence of caruncle/plica
  • Fibrotic/scarred tear ducts , destruction of goblet cells (-> dry eye)
  • Cornea: Epithelial defects, dellen, ulcers, perforation, vascularisation, keratinisation/conjunctivalisation
  • Symblepharon (adhesion of the bulbar to the palpebral conjunctiva)
  • Ankyloblepharon (adhesion between upper and lower lid)
  • Entropium with trichiasis

Foster’s Classification System

  • Stage I: Early stage with minimal changes (subepithelial fibrosis, chronic conjunctivitis)
  • Stage II: Fornix shortening
  • Stage III: Symblepharon
  • Stage IV: Ankyloblepharon


  • Biopsy (histology + immunofluorescence: linear deposits of IgG, IgA, and/or complement C3 along the basement membrane)


  • Local lubrication (preservative-free), autologous autologous serum eye drops
  • Local steroids or topical cyclosporine (Ikervis) against local inflammation (usually not sufficient)
  • Systemic therapy/immunosuppressants
    • Dapsone
    • Methotrexate
    • Azathioprine (Imurek)
    • Mycophenolate mofetil
    • Cyclophosphamide
  • For dosages and recommended lab tests, see treatment recommendations from rheuma-net.ch

Differential Diagnoses/ Pseudopemphigoid

  • Drug-induced: chronic topical therapy (including Pilocarpine, Epinephrine, Timolol, Idoxuridine)
    • No progression after discontinuation of medications, clinical changes especially in the inferior fornix
  • Post-inflammatory scarring
  • Autoimmune diseases: Sarcoidosis, scleroderma, lichen planus, Stevens-Johnson, dermatitis herpetiformis, epidermolysis bullosa, atopic blepharoconjunctivitis, graft-vs-host-disease
  • Trauma: chemical injuries, burns
  • Severe blepharoconjunctivitis e.g. in rosacea
  • IgA dermatosis (clinically similar presentation!)