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A 79-year-old female presented with a 3-year history of trichiasis and red eyes (figure 1A). Multiple operations for entropion and trichiasis had been carried out previously, and a conjunctival biopsy investigating mucous membrane pemphigoid (MMP), had been negative. Her right eye was blind due to retinal detachment and glaucoma. Acuity was light perception in the right eye (OD) and 6/12 in the left eye (OS). There was active inflammation and bilateral inferior symblepharon, left upper lid entropion and trichiasis. There were no systemic features of MMP.
Conjunctival and buccal mucosa biopsies were undertaken. Serum was tested for indirect inmunofluorescence. Conjunctival and serum immunofluorescence were negative, but buccal mucosa showed linear IgG and IgA staining along the basement membrane zone (BMZ), consistent with MMP.
Oral prednisolone 1 mg/kg/day on a tapering course over 6 weeks, and mycophenolate 1 g twice daily were commenced. Nine months later, left upper lid entropion surgery was performed with excellent results.
Questions (for answers see 537)
What investigations are needed to identify the cause of cicatrising conjunctivitis?
Describe how to do conjunctival and buccal biopsies?
How would you manage the patient if you suspect ocular MMP, but all immunopathology …
Contributors VPJS initiated the concept of the paper, drafted and revised the paper. She is guarantor. AEG drafted the paper. JS initiated the concept of taking buccal biopsies in ocular patients who have no mouth symptoms or signs, provided her own unpublished data for the paper discussion, and revised the draft paper. JD's patients were used for JS's data, and he commented on the draft paper.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; internally peer reviewed.