# Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature

## TL;DR

A 12-year-old girl with rare ocular mucous membrane pemphigoid showed successful treatment with methotrexate after initial therapies failed.

## Contribution

This is the first reported case of pediatric ocular MMP in a Hispanic patient presenting with peripheral ulcerative keratitis and responding to methotrexate.

## Key findings

- The patient's diagnosis was confirmed via conjunctival biopsy and immunofluorescence showing basement membrane zone deposits.
- Methotrexate successfully controlled disease activity after initial treatments with dapsone and prednisone failed.
- After three years of follow-up, the patient remained free of disease activity.

## Abstract

The purpose of this study was to describe the case of a pediatric patient diagnosed with mucous membrane pemphigoid (MMP) with exclusive ocular involvement presenting at diagnosis with peripheral ulcerative keratitis and provide a review of the literature.

A 12-year-old girl presented with cicatricial conjunctivitis and peripheral ulcerative keratitis (PUK). A conjunctival biopsy and direct immunofluorescence revealed linear deposits of IgG, IgM, and C3 at the basement membrane zone, confirming a diagnosis of ocular MMP.

The patient was initially treated with dapsone 25 mg/day and prednisone 40 mg/day along with topical treatment including cyclosporine 0.05%, loteprednol etabonate 0.05%, and sodium hyaluronate 0.15% and trehalose 3%. Inflammation persisted as an increase in the extension of symblepharon was documented. Subsequently, dapsone was switched to oral methotrexate 15 mg/week and prednisone was successfully tapered to 5 mg/day. After three years of follow-up, disease activity remained quiescent.

Pediatric mucous membrane pemphigoid with ocular involvement is a rare condition of which few reports have been published, resulting in scarce information regarding its clinical course and response to treatment. We report the first case observed in a Hispanic patient, opening with peripheral ulcerative keratitis, and responding successfully to methotrexate.