Cicatricial conjunctivitis refers to inflammation of the conjunctiva which has led to scarring. It can be used to describe a wide number of conditions from chemical burns to infections such as trachoma, and systemic diseases such as sarcoidosis and Stevens-Johnson syndrome. The presence of a cicatrix (conjunctival scar) should be suspected in trichiasis, entropion, symblepharon and keratinisation.
Pathology
- These diseases are typically bilateral and progressive.
- The essential pathology is inflammation which leads to loss of goblet cells → failure of ocular surface integrity → limbitis and limbal stem cell failure → keratopathy and scarring
Symblepharon of the lower conjunctiva By Ajeesh kumar, CC BY-SA 4.0.
Ocular Mucous Membrane Pemphigoid
A chronic blistering type 2 hypersensitivity reaction of the mucosal surfaces. Suspect in severe bilateral cases of papillary conjunctivitis with evidence of cicatrisation and systemic cutaneous involvement.
Pathology
- Linear deposits of IgA, IgG and complement in the basement membranes of mucosal surfaces. This can be seen on conjunctival autofluorescence.
- The antibodies target hemidesmosomes and components of the basement membranes.
Management
In general, topical steroids and doxycycline are administered. Disease-modifying treatment is stepwise:
- Mild → dapsone
- Moderate → mycophenolate, methotrexate or azathioprine
- Severe → IV methylprednisolone and/or cyclophosphamide or rituximab long term (se. pulmonary toxicity)
Erythema Multiforme, SJS and TEN
These conditions can be thought of as a spectrum of diseases where TEN is the most severe.
Pathology
- Inflammation of the vessels of the mucous membranes and skin, driven by type 4 hypersensitivity to a variety of triggers.
- Triggers include: drugs (sulfonamides, allopurinol and AEDs) and infections such as HSV
Presentation
- Acutely unwell with target lesions, bullae and mucous membrane inflammation.
- Nikolsky sign - sloughing sheets of skin
Management
- Management in the acute phase is with steroids and expert help with a burns unit should be sought, particularly in cases of TEN.
- In the chronic phase, management is stepwise and follows the same structure as for ocular mucous membrane pemphigoid (above).
