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

Abstract Purpose 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. Methods A 12-year-old girl...

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Main Authors: Eugenia M. Ramos-Dávila, Raul E. Ruiz-Lozano, Alejandro Rodríguez-García, Carlos Álvarez-Guzmán
Format: Article
Language:English
Published: SpringerOpen 2025-05-01
Series:Journal of Ophthalmic Inflammation and Infection
Online Access:https://doi.org/10.1186/s12348-025-00480-y
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author Eugenia M. Ramos-Dávila
Raul E. Ruiz-Lozano
Alejandro Rodríguez-García
Carlos Álvarez-Guzmán
author_facet Eugenia M. Ramos-Dávila
Raul E. Ruiz-Lozano
Alejandro Rodríguez-García
Carlos Álvarez-Guzmán
author_sort Eugenia M. Ramos-Dávila
collection DOAJ
description Abstract Purpose 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. Methods 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. Results 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. Conclusions 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.
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spelling doaj-art-dd1b815c8e894d8c9ca196c9006d1d392025-08-20T03:22:11ZengSpringerOpenJournal of Ophthalmic Inflammation and Infection1869-57602025-05-011511710.1186/s12348-025-00480-yChildhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literatureEugenia M. Ramos-Dávila0Raul E. Ruiz-Lozano1Alejandro Rodríguez-García2Carlos Álvarez-Guzmán3Tecnologico de Monterrey, Escuela de Medicina y Ciencias de la SaludBascom Palmer Eye Institute, University of MiamiTecnologico de Monterrey, Escuela de Medicina y Ciencias de la SaludTecnologico de Monterrey, Escuela de Medicina y Ciencias de la SaludAbstract Purpose 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. Methods 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. Results 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. Conclusions 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.https://doi.org/10.1186/s12348-025-00480-y
spellingShingle Eugenia M. Ramos-Dávila
Raul E. Ruiz-Lozano
Alejandro Rodríguez-García
Carlos Álvarez-Guzmán
Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature
Journal of Ophthalmic Inflammation and Infection
title Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature
title_full Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature
title_fullStr Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature
title_full_unstemmed Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature
title_short Childhood-onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis: a case report and review of the literature
title_sort childhood onset ocular mucous membrane pemphigoid presenting with peripheral ulcerative keratitis a case report and review of the literature
url https://doi.org/10.1186/s12348-025-00480-y
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