Childhood Mucous Membrane Pemphigoid: A Case With Exclusively Ocular Involvement

Abstract

Purpose: To report a case of mucous membrane pemphigoid (MMP) occurring in childhood with exclusively ocular involvement.

Methods: Case report.

Results: A 12-year-old girl presented with mild bilateral conjunctival cicatrization, symblepharon, and peripheral inflammatory keratitis. No other mucosal or cutaneous involvement was present. The findings of an initial conjunctival biopsy with immunofluorescence study were negative for MMP. After a few recurrences of mild corneal and conjunctival inflammation, a second conjunctival biopsy was performed at a different site in the same eye, 30 months later. Only then was the diagnosis of MMP confirmed using positive direct immunofluorescence. The results of a systemic investigation for autoimmune vasculitis were negative. Therapy with dapsone was introduced, and a good response was obtained.

Conclusions: MMP is an extremely rare condition in children. This case is the second one reported with exclusively ocular involvement. Despite its rarity in the pediatric age group, MMP should be considered in the differential diagnosis of chronic conjunctival inflammation and fibrosis. Furthermore, a single negative biopsy should not exclude this diagnosis in a suspected clinical case, even in children. When a high degree of suspicion is present, a repeat biopsy may prove useful.