Anterior segment - ocular surface
Ocular Cicatricial Pemphigoid
Pathophysiology
- Autoantibodies directed against bullous pemphigoid antigen II (BP180) in the basement membrane zone
Clinical features
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Generally bilateral
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Inferior forniceal depth of less than 8mm
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Early findings
- Acute papillary conjunctivitis
- Medial canthal keratinization
- Trichiasis
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Symblepharon
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Ankyloblepharon
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Late stage: secondary corneal scarring
Hot Topic
Causes of symblepharon
- OCP
- Trachoma
- Chemical injury
- Stevens-Johnsons syndrome/Toxic epidermal necrolysis
Histology
- Subepithelial bullae and a mix of chronic inflammatory cells, sometimes a predominance of plasma cells which stain CD138
Hot Topic
Conjunctival biopsy is required for definitive diagnosis but must be cautiously performed as even minor surgery can trigger deterioration in the disease
Management
- Optimisation of the ocular surface: lid management, lubricants
- Dapsone
- Steroids for the acute phase: topical and systemic
- Further immunosuppression
- Cautious lid procedures to manage complications such as trichiasis