AtlasVesiculobullousMucous Membrane Pemphigoid

Vesiculobullous Diseases

Mucous Membrane Pemphigoid

aka MMP · Cicatricial Pemphigoid · Benign Mucous Membrane Pemphigoid

Chronic autoimmune subepithelial blistering disease affecting mucous membranes with scarring potential, especially in eyes.

Split
Subepithelial
DIF
Linear IgG/C3 at BMZ
Risk
Ocular scarring

Red Flags

  • ·Ocular involvement
  • ·Laryngeal involvement

§ overviewOverview

Autoimmune disease with autoantibodies against basement membrane zone proteins causing subepithelial blisters.

§ icdICD Classification

L12.1

§ etiologyEtiology

  • 01Autoantibodies against BP180, BP230, laminin-332, laminin-311, type VII collagen

§ epidemiologyEpidemiology

Elderly, F > M. Oral mucosa involved in 85%.

§ pathogenesisPathogenesis

Autoantibodies bind BMZ → complement activation → neutrophil chemotaxis → subepithelial separation.

§ clinicalClinical Features

  • 01Intact blisters (more common than pemphigus due to thicker roof)
  • 02Desquamative gingivitis
  • 03Positive Nikolsky sign (may be negative)
  • 04Ocular: symblepharon, entropion, blindness
  • 05Other sites: nose, pharynx, larynx, genitalia

§ differentialDifferential Diagnosis

  • 01Pemphigus vulgaris
  • 02Bullous pemphigoid
  • 03Erythema multiforme
  • 04Lichen planus

§ histopathHistopathology

  • 01Subepithelial separation
  • 02Intact basal cells on floor of blister
  • 03Mixed inflammatory infiltrate

§ investigationsInvestigations

  • 01Perilesional biopsy for DIF
  • 02Serum ELISA/IIF for circulating antibodies

§ ihcIHC / Special Stains

  • 01DIF: Linear IgG/C3 at BMZ

§ treatmentTreatment

  • 01Mild oral: topical corticosteroids, tacrolimus
  • 02Moderate: dapsone, tetracycline + niacinamide
  • 03Severe/ocular: systemic steroids, azathioprine, mycophenolate, rituximab

§ complicationsComplications

  • 01Symblepharon, trichiasis, blindness
  • 02Laryngeal stenosis

§ prognosisPrognosis

Chronic; ocular scarring is major morbidity.

§ examKey Examination Points

  • 01Desquamative gingivitis common
  • 02Ocular involvement → scarring → blindness
  • 03DIF: linear BMZ

§ revisionQuick Revision Summary

  • 01Subepithelial blisters
  • 02Linear IgG BMZ
  • 03Ocular scarring risk

§ vivaBDS Viva Questions

  • 01Differentiate MMP from pemphigus vulgaris.
  • 02What is symblepharon?
  • 03Describe the DIF findings.

§ mcqsMCQs — Assessment (3)

Question 1

MMP shows on DIF:

Question 2

Most concerning complication of MMP:

Question 3

Histology shows:

References

  1. Neville BW. Oral & Maxillofacial Pathology, 4e
  2. Chan LS. J Am Acad Dermatol 2002

Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.