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Mucous Membrane Pemphigoid (Ocular Cicatricial Pemphigoid)

CHRONIC — Adnexa

Symptoms

insidious onset of chronic dryness, redness, and burning sensation

foreign body sensation, tearing, and blepharospasm

progressive bilateral involvement typically in patients over 55

decreased vision and photophobia in advanced stages

Clinical Findings

Progressive cicatrizing conjunctivitis

  • chronic scarring that starting as a non-specific "red eye"
  • symblepharon (hallmark): fibrous adhesions between bulbar and palpebral conjunctiva
  • fornix foreshortening: measured depth <11 mm (serial measurement tracks progression)
  • ankyloblepharon: fusion of the lids to the globe in end-stage disease

Lid & corneal sequelae

  • trichiasis and entropion (misdirected lashes and inward lid rotation)
  • keratinization of the conjunctiva and severe goblet cell loss
  • limbal stem cell deficiency and eventual corneal opacification

Extraocular mucosa

  • desquamative gingivitis (red, sloughing gums) is the most common finding
  • bullae and ulcers of the oral, nasopharyngeal, or esophageal mucosa

Etiology

Autoimmune

  • type II hypersensitivity: autoantibodies against basement membrane proteins (BP180, laminin-332)
  • drug-induced: long-term use of topical glaucoma drops (pilocarpine, epinephrine)

Red Flags

progressive symblepharon formation (requires aggressive systemic immunosuppression)

esophageal or tracheal strictures (life-threatening systemic involvement)

corneal melting or epithelial breakdown

failure to respond to initial dapsone therapy

Investigations

Clinical assessment

  • forniceal measurement: pull lower lid down while patient looks up to expose symblepharon
  • check oral and nasal mucosa for ulcers

Diagnostics

  • conjunctival biopsy with direct immunofluorescence (DIF): from PERILESIONAL (adjacent uninvolved) tissue
  • DIF signature: linear deposition of IgG, IgA, or C3 along the basement membrane
  • indirect immunofluorescence on serum if biopsy is inconclusive

Treatment

Systemic (primary)

  • dapsone: first-line for mild/moderate disease (monitor for hemolysis)
  • steroid-sparing agents: methotrexate, mycophenolate mofetil, or cyclophosphamide
  • biologic therapy: rituximab or ivig for severe, progressive cases

Ocular surface

  • preservative-free artificial tears, autologous serum, and moisture goggles
  • bandage or scleral lenses to protect the cornea

Surgical

  • entropion or trichiasis repair only when the disease is medically quieted
  • note: surgery during active inflammation significantly accelerates scarring

Clinical Pearl

Shortening of the inferior fornix is the earliest ocular sign of MMP — by the time you see symblepharon and cicatrization, the disease is advanced; systemic immunosuppression must start before surgical repair.

Differential Diagnosis

  • stevens–johnson syndrome (acute onset, history of drug reaction)
  • atopic keratoconjunctivitis
  • chronic chemical burn sequelae
  • trachoma

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