Cicatrising Conjunctivitis: Mucous Membrane Pemphigoid and SJS/TEN
Key Takeaways
Document fornix shortening and symblepharon; apparent quietness does not prove scarring has stopped.
Negative DIF does not exclude ocular MMP, and specimen transport must match the test.
Progressive ocular MMP commonly needs systemic immune control as well as local symptom relief.
Chronic SJS injury may be driven by lid keratinisation and mechanical trauma as well as tear deficiency.
Recognise progressive scarring
Cicatrising conjunctivitis means conjunctival inflammation with scarring. Its consequences include shortening of fornices, adhesions between bulbar and palpebral conjunctiva (symblepharon), lid malposition, tear deficiency, limbal failure and corneal damage. The patient may complain of irritation long before advanced adhesions are obvious. Examine and compare all fornices and document progression with photographs and measurements; a quiet-looking conjunctiva does not always mean that the disease has stopped.
The differential includes ocular mucous membrane pemphigoid, Stevens–Johnson syndrome/toxic epidermal necrolysis, chronic medication toxicity, chemical injury, trachoma, graft-versus-host disease and other inflammatory conditions. A focal unilateral process can suggest neoplasia or local injury. Review all topical drops, previous surgery and systemic mucosal symptoms. Do not label every shortened fornix as pemphigoid without considering competing causes.
Ocular mucous membrane pemphigoid
Mucous membrane pemphigoid (MMP) is an autoimmune blistering disorder targeting epithelial basement-membrane-zone structures. Ocular disease can be associated with oral, nasal, pharyngeal, genital or other mucosal involvement. Ask about painful oral lesions, swallowing difficulty and airway symptoms and involve the relevant specialists; airway disease can be serious even when the main presenting complaint is ocular irritation.
Conjunctival or other appropriate mucosal biopsy for direct immunofluorescence can show linear immunoreactant deposition along the basement membrane. Plan site and transport with the laboratory, avoiding the assumption that a formalin-fixed histology specimen can also provide a valid DIF result. A negative test does not exclude ocular MMP. Repeat or alternative-site investigation and exclusion of other causes may be necessary. The European S3 guideline specifically addresses immunopathology-negative ocular disease.
Topical lubricants and short courses of local anti-inflammatory treatment can improve symptoms but do not reliably prevent progressive immune-mediated scarring. Active progressive ocular MMP usually requires systemic immunomodulatory treatment with ophthalmology and dermatology/rheumatology coordination. Agent selection depends on severity, comorbidity and monitoring. Do not perform elective lid or surface reconstruction through uncontrolled inflammation when stabilisation is possible. Conversely, a threatened cornea may need protective measures urgently while systemic treatment is organised.
SJS and toxic epidermal necrolysis
Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions, often associated with medication exposure. Systemic stabilisation, withdrawal of the suspected trigger and acute medical care take priority. Ocular severity does not perfectly track the percentage of skin involvement. Early ophthalmic examination must inspect lid margins, conjunctiva, fornices and corneal epithelium, including hidden defects and pseudomembranes.
Acute management aims to preserve epithelial surfaces and prevent adhesions. Appropriate lubrication, removal of damaging debris, carefully selected topical therapy and early specialist consideration of amniotic membrane are relevant. Membrane coverage must address the involved surfaces; a small central corneal device does not protect damaged lid margins or the entire fornix. Timing and technique depend on the actual epithelial injury, and membrane placement is not a substitute for systemic care.
In the chronic phase, lid-margin keratinisation, trichiasis, dry eye, symblepharon and limbal stem-cell failure may mechanically damage the cornea. Treat the driver rather than escalating lubricants alone. Selected management includes mucous-membrane grafting, lash or lid repair, scleral devices and surface reconstruction, each with its own infection and healing risks. A clear graft placed in a severely dry, keratinised and inflamed environment has poor prospects unless those problems are addressed.
Distinguishing mechanism and stage
| Feature | Ocular MMP | SJS/TEN-associated disease |
|---|---|---|
| Typical course | Chronic progressive immune-mediated scarring | Acute mucocutaneous episode followed by variable chronic damage |
| Diagnostic evidence | Clinical phenotype plus DIF/serology and exclusion of mimics | History and systemic/ocular features of the acute reaction |
| Major ongoing driver | Active inflammation and fibrosis | Residual surface failure, keratinisation, lid trauma and sometimes inflammation |
| Key management question | Is systemic immune control adequate? | Which damaged surface or lid structure is driving current injury? |
These distinctions are useful but not absolute. A person with an old acute reaction can still have active inflammation, and a person with MMP can have severe mechanical damage after immune control. Assess both activity and accumulated structural injury at every visit.
A scarring case
An older adult using several glaucoma drops has bilateral irritation and shortened inferior fornices. Review the medication history, inspect upper fornices and oral mucosa, document scarring and seek specialist assessment. Medication-related pseudopemphigoid is a possibility, but stopping a suspected drop without an alternative pressure plan can harm the optic nerve. Arrange investigations and coordinate substitutions, then judge progression over time.
A patient recovering from SJS with a persistent corneal defect needs assessment of sensation, lid closure, lid-margin keratinisation, tear function and limbal status, as well as infection. Protective treatment can be urgent even if the systemic eruption has healed. The EBO external-disease syllabus includes these cicatrising disorders because preventing further scarring and rebuilding a viable surface are different clinical tasks.
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