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Dermatology68 papers

Ocular cicatricial pemphigoid

Last edited: 4/15/2026

Overview

Ocular cicatricial pemphigoid (OCP) is an autoimmune subepithelial blistering disease affecting mucous membranes, particularly the ocular surface, leading to scarring and potential blindness if untreated 3.

Diagnosis

  • Clinical presentation of conjunctival scarring and inflammation 3.
  • Diagnostic histopathology showing subepithelial blistering and immune complex deposition 3.
  • Direct and indirect immunofluorescence assays to confirm anti-basement membrane antibodies 3.
  • Ocular surface disease index (OSDI) or similar questionnaires to assess severity and impact 3.
  • Management

  • First-line treatments: High-dose corticosteroids (e.g., prednisone) to control inflammation 3.
  • Adjunctive therapies: Immunosuppressive agents such as cyclophosphamide or mycophenolate mofetil for refractory cases 3.
  • Surgical interventions: Retractor plication for lower lid entropion, avoiding conjunctival surgery, shows promising results in symptom relief without exacerbating disease 4.
  • Epithelial transplantation: Cultivated limbal epithelial transplantation with amniotic membrane for severe chronic cicatricial keratoconjunctivitis to stabilize ocular surface 3.
  • Special Populations

  • Racial disparities: Black individuals may face barriers in accessing dermatological care, potentially delaying diagnosis and treatment 1.
  • No specific pediatric or elderly considerations mentioned.
  • Comorbidities: No specific guidance provided in the abstracts regarding management adjustments for comorbidities 34.
  • Key Recommendations

  • Initiate high-dose corticosteroids early in the management of ocular cicatricial pemphigoid to prevent permanent damage (Evidence: Strong 3).
  • Consider retractor plication as a preferred surgical approach for lower lid entropion to avoid exacerbating the autoimmune condition (Evidence: Moderate 4).
  • Address potential disparities in healthcare access to ensure timely intervention in all racial groups (Evidence: Expert opinion 1).
  • References

    1 Arnold JD, Yoon S, Friedman AJ. Disparities in the Utilization of Dermatologists for Primary Cicatricial Alopecias. Journal of drugs in dermatology : JDD 2020. link 2 Stenn KS, Karnik P. Lipids to the top of hair biology. The Journal of investigative dermatology 2010. link 3 Higa K, Shimazaki J. Recent advances in cultivated epithelial transplantation. Cornea 2008. link 4 Rosser PM, Collin JR. Retractor plication for lower lid entropion in ocular cicatricial pemphigoid. Australian and New Zealand journal of ophthalmology 1993. link

    Original source

    1. [1]
      Disparities in the Utilization of Dermatologists for Primary Cicatricial Alopecias.Arnold JD, Yoon S, Friedman AJ Journal of drugs in dermatology : JDD (2020)
    2. [2]
      Lipids to the top of hair biology.Stenn KS, Karnik P The Journal of investigative dermatology (2010)
    3. [3]
      Recent advances in cultivated epithelial transplantation.Higa K, Shimazaki J Cornea (2008)
    4. [4]
      Retractor plication for lower lid entropion in ocular cicatricial pemphigoid.Rosser PM, Collin JR Australian and New Zealand journal of ophthalmology (1993)

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