Symblepharon

Mucous membrane pemphigoid with inferior symblepharon (Wills Eye Manual, Fig. 5.10.1)

Causes (Differential Diagnosis)

Key note: Symblepharon following a single insult (burn, SJS) tends to be stable, while that associated with MMP/OCP is typically progressive.

• The Wills Eye Manual, p. 401; p. 356
• Kanski's Clinical Ophthalmology 10th ed., p. 648

Staging (in the context of MMP/OCP)

Foster's Classification (most widely used)

Mondino's Classification (based on inferior forniceal depth loss)

• Kanski's Clinical Ophthalmology 10th ed., p. 621-638

Clinical Features

• Critical: Inferior symblepharon - linear conjunctival folds connecting palpebral to bulbar conjunctiva; best seen by pulling down the lower lid during upgaze
• Foreshortening and tightening of the inferior fornix
• Palpebral conjunctival scarring on eyelid eversion
• Secondary: SPK (superficial punctate keratopathy), bacterial conjunctivitis, corneal ulcer

• Severe dry eye (goblet cell and lacrimal gland destruction)
• Entropion, trichiasis, distichiasis
• Corneal opacification with pannus and neovascularization
• Ankyloblepharon (upper and lower lids fused at the outer canthus)
• Limitation of ocular motility
• Total symblepharon with corneal opacification (end-stage)

• The Wills Eye Manual, pp. 355-357
• Kanski's Clinical Ophthalmology 10th ed., pp. 648-675

Workup

1. History - long-term topical medications? acute systemic illness? recent systemic drug use?
2. Skin and mucous membrane examination
3. Slit-lamp exam - check inferior fornix depth, evert lids for palpebral scarring, check IOP
4. Gram stain and culture if secondary bacterial infection suspected
5. Conjunctival biopsy (using Michel's transport medium) for:
   • Direct immunofluorescence: linear IgG, IgA, or C3 at the basement membrane zone (confirmatory for MMP)
   • Indirect immunofluorescence for circulating antibodies
6. Multidisciplinary consults as appropriate (dermatology, oculoplastics, ENT, GI, pulmonology)

Treatment

Supportive / Ocular Surface

• Preservative-free artificial tears 4-10x/day; ointment b.i.d.-q.i.d. and at night; autologous serum drops 20-50%
• Scleral lenses to maintain ocular surface integrity
• Moisture chamber goggles
• Punctal occlusion (if puncta not already scarred closed)
• Treat blepharitis: lid hygiene, warm compresses, topical erythromycin; oral doxycycline for anti-inflammatory effect

Medical (Immunosuppression)

• Topical steroids - for acute flares only; use with caution (risk of corneal melting)
• Systemic steroids (prednisone 60 mg/day) - for acute exacerbations; most effective combined with immunomodulators
• Immunosuppressives (for progressive disease): mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab, IV immunoglobulin
• Dapsone - start 25 mg/day; increase by 25 mg every 4-7 days; target 100-150 mg/day; maintain for months then taper slowly
  • Caution: check G-6-PD before starting; monitor CBC weekly during dose escalation (hemolysis risk)

Surgical

• Entropion correction; cryotherapy or electrolysis for trichiasis - only when inflammation is absent
• Mucous membrane grafts (buccal or amniotic membrane) to reconstruct fornices
• Keratoprosthesis for end-stage disease with good posterior segment function (guarded long-term prognosis)

Follow-up

• Every 1-2 weeks during acute exacerbations
• Every 1-6 months during remission
• The Wills Eye Manual, pp. 357-358

Symblepharon

Causes / Differential Diagnosis

Key clinical point: Symblepharon following a single acute insult (burn, SJS) tends to be stable. Symblepharon in MMP/OCP is typically progressive and requires aggressive systemic immunosuppression.

• The Wills Eye Manual, p. 401; p. 356
• Kanski's Clinical Ophthalmology 10th ed., p. 648

Staging Systems (in MMP/OCP)

Foster's Classification (most widely used)

Mondino's Classification (based on inferior forniceal depth loss)

• Kanski's Clinical Ophthalmology 10th ed., p. 621-638

Clinical Features

Symptoms

• Dryness, redness, foreign body sensation, burning
• Blepharospasm, photophobia, tearing
• Decreased vision

Ocular Signs

• Inferior symblepharon - linear conjunctival folds connecting palpebral to bulbar conjunctiva (best seen by pulling down the lower lid during upgaze)
• Foreshortening and tightness of the inferior fornix
• Palpebral conjunctival scarring on eyelid eversion

Fig. 5.10.1 - Mucous membrane pemphigoid with symblepharon (Wills Eye Manual)

Fig. 6.17 - Conjunctivitis in ocular cicatricial pemphigoid: A=early fibrosis; B=symblepharon; C=severe fibrosis + symblepharon; D=ankyloblepharon (Kanski's Clinical Ophthalmology 10th)

• Conjunctiva: papillary conjunctivitis, goblet cell destruction → severe dry eye; flattening of plica; caruncular keratinization
• Eyelids: entropion, trichiasis/distichiasis, aberrant lashes, blepharitis, keratinization of lid margin
• Cornea: SPK, epithelial defects, peripheral vascularization, keratinization, conjunctivalization (stem cell failure), stromal ulcers, opacification
• End-stage: total symblepharon, ankyloblepharon (lid-to-lid fusion), limitation of ocular motility, corneal opacification

Systemic Features (in MMP)

• Mucous membrane blistering: oral (most common - desquamative gingivitis), oropharynx, esophagus, vagina, urethra, anus
• Oesophageal and laryngeal strictures (severe)
• Skin lesions (less common, ~25%): tense blisters and erosions of head/neck, groin, extremities
• The Wills Eye Manual, pp. 354-358
• Kanski's Clinical Ophthalmology 10th ed., pp. 644-740

Workup

1. History - long-term topical medications? past acute systemic illness? recent drug exposure (sulfa, phenytoin, penicillin)?
2. Full skin and mucous membrane examination
3. Slit-lamp exam - forniceal foreshortening, inferior symblepharon (lower lid traction during upgaze), evert lids for palpebral scarring; check IOP
4. Gram stain and culture if secondary bacterial infection suspected
5. Conjunctival/mucous membrane biopsy for:
   • Direct immunofluorescence: linear IgG, IgA, or C3 at basement membrane zone (confirmatory for MMP)
   • Indirect immunofluorescence for circulating antibodies
   • Use Michel's transport medium for specimen transport
6. Multidisciplinary consults: dermatology, oculoplastics, ENT, gastroenterology, pulmonology

Treatment

Ocular Surface / Supportive

• Preservative-free artificial tears 4-10x/day; high-viscosity ointment at night; autologous serum drops 20-50% q.i.d.
• Scleral lenses to maintain ocular surface integrity
• Moisture chamber goggles/glasses
• Punctal occlusion (if not already scarred)
• Treat blepharitis: lid hygiene, warm compresses, erythromycin ointment t.i.d.; oral doxycycline for anti-inflammatory effect
• Topical ciclosporin or tacrolimus as adjuncts; retinoic acid to reduce keratinization

Medical (Immunosuppression for MMP/OCP)

"Any detectable inflammatory activity should be suppressed." - Kanski's, p. 686

Surgical (only when inflammation is controlled)

• Entropion correction; cryotherapy or electrolysis for trichiasis
• Subconjunctival mitomycin C and/or steroid injection (temporizing measure)
• Mucous membrane grafts (buccal mucosa or amniotic membrane) to reconstruct fornices and prevent further symblepharon
• Contact lens use (with caution) to protect cornea from aberrant lashes
• Keratoprosthesis for end-stage eyes with good posterior segment function (prognosis guarded)

Important: Even seemingly trivial surgical intervention can trigger disease exacerbation in active OCP. Surgery should only be performed under systemic steroid cover when inflammation is absent.

Follow-up

• Every 1-2 weeks during acute exacerbations
• Every 1-6 months during remission