Management of patients with confirmed and presumed mucous membrane pemphigoid undergoing entropion repair.

Purpose: To describe the outcomes and medical management necessary to achieve successful lid surgery in patients with biopsy-confirmed and presumed mucous membrane pemphigoid.

Design: Retrospective, interventional case series.

Methods: We included patients with positive biopsy results and cases with a typical clinical active bilateral presentation with negative biopsy results but classic features. We identified 11 operated eyes of 7 patients with lid malposition resulting from mucous membrane pemphigoid, particularly cicatricial entropion, that required surgical correction. Complete ophthalmologic history and examination were performed. The main outcome measures were control of ocular inflammation, progression of disease, and surgical success.

Results: A bandage lens was used in 8 (72.7%) eyes to protect the cornea while immunosuppression and control of disease activity were achieved. Control of ocular inflammation before lid surgery was achieved in all cases. Immunosuppressive treatment before lid surgery was used in all cases for a mean of 15.1 months (range, 8.2 to 33.1 months) and after surgery for a mean of 6.6 months (range, 3.0 to 11.2 months). The oral immunosuppressive drugs used were mycophenolate and cyclophosphamide. Prednisone was used concomitantly in 4 (57%) patients. Full surgical success was achieved in all patients, with 1 patient requiring a second intervention because of residual disease. The mean postoperative follow-up period was 20.8 months (range, 6.0 to 30.5 months).

Conclusions: Successful entropion repair in patients with mucous membrane pemphigoid can be achieved if control of inflammation is attained before the procedure. Ocular surface protection while achieving disease control is essential in the management of these patients.