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Mucous Membrane Pemphigoid (Cicatricial Pemphigoid)

Chronic autoimmune subepithelial blistering disease primarily affecting mucous membranes with scarring.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Mucous Membrane Pemphigoid (Cicatricial Pemphigoid)?

Mucous membrane pemphigoid (MMP), formerly cicatricial pemphigoid, is a chronic autoimmune subepithelial blistering disease caused by autoantibodies against multiple basement membrane zone antigens (BP180, BP230, laminin 332, type VII collagen, alpha 6 beta 4 integrin).

It primarily affects mucous membranes (oral, ocular, nasopharyngeal, laryngeal, esophageal, genital, anal), with skin involvement in only 25-30% of patients. Repeated blistering and erosions cause progressive scarring that can lead to symblepharon, ankyloblepharon, blindness, laryngeal stenosis and dysphagia.

Anti-laminin 332 MMP is associated with an increased risk of underlying solid malignancy and warrants thorough cancer screening at diagnosis.

Symptoms

Recurrent oral mucosa erosions and gingivitis
Conjunctival redness, scarring and symblepharon
Photophobia and burning eyes
Hoarseness from laryngeal involvement
Dysphagia from esophageal strictures
Genital and anal mucosa erosions
Skin tense blisters in 25-30%
Progressive vision loss without treatment

Risk Factors

Older age (60-80 years)
Female sex (2:1)
HLA-DQB1*0301 association
Anti-BP180, anti-BP230 autoantibodies
Anti-laminin 332 antibodies (cancer association)
Anti-type VII collagen variant
Use of certain medications (penicillamine, captopril)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent oral mucosa erosions or gingivitis lasting more than 1 month
  • Recurrent conjunctival redness or symblepharon
  • Hoarseness with mucosal blistering
  • Vision changes with eye redness
  • Dysphagia with mucosal disease
  • Need for biopsy with direct immunofluorescence

Treatment Methods

01
Multidisciplinary care (dermatology, ophthalmology, ENT, dentistry)
02
Topical and systemic corticosteroids
03
Dapsone for mild-moderate disease
04
Cyclophosphamide for severe ocular disease
05
Rituximab for refractory cases
06
Mycophenolate mofetil or azathioprine
07
IVIG in steroid-resistant disease
08
Cancer screening for anti-laminin 332 variant

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.