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Mucous membrane pemphigoid and pseudopemphigoid.

Jennifer E Thorne1, Grant J Anhalt, Douglas A Jabs

  • 1Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.

Ophthalmology
|January 9, 2004
PubMed
Summary
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Mucous membrane pemphigoid (MMP) often presents with systemic symptoms and carries a risk of ocular involvement, even initially without eye disease. Differentiating MMP from pseudopemphigoid requires immunohistologic evaluation due to similar clinical features.

Area of Science:

  • Ophthalmology
  • Dermatology
  • Immunology

Background:

  • Mucous membrane pemphigoid (MMP) is a rare autoimmune blistering disease affecting mucous membranes.
  • Ocular involvement is a significant concern in MMP, potentially leading to severe visual impairment.
  • Pseudopemphigoid shares clinical similarities with MMP, complicating diagnosis.

Purpose of the Study:

  • To delineate the clinical characteristics of patients diagnosed with mucous membrane pemphigoid (MMP) and pseudopemphigoid.
  • To assess the risk of developing ocular disease in patients initially presenting with extraocular MMP.

Main Methods:

  • Retrospective cohort study of 280 patients evaluated for ocular MMP.
  • Data collected prospectively and via retrospective chart review from January 1985 to December 2001.

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  • Diagnosis confirmed by direct immunofluorescence or circulating autoantibodies to the epithelial basement membrane zone (BMZ).

    • Main Results:

    • Extraocular disease was present in 82.4% of patients with ocular MMP.
    • Patients with initial extraocular MMP faced a cumulative risk of 22% for ocular involvement within 5 years.
    • Common causes of pseudopemphigoid included glaucoma medications (28.3%) and rosacea blepharoconjunctivitis (20.0%).

    Conclusions:

    • Ocular MMP frequently co-occurs with systemic manifestations.
    • Extraocular MMP necessitates vigilant monitoring for the potential development of ocular disease.
    • Immunohistologic evaluation is crucial for distinguishing MMP from pseudopemphigoid due to overlapping clinical presentations.