Necrotizing Anterior Scleritis in a Woman with Terrien’s Marginal Degeneration: A Case Report

María Alejandra Fonseca-Mora,William Rojas-Carabali,Paula Tatiana Muñoz-Vargas,Miguel Cuevas,Alejandra De-La-Torre,Juliana Reyes-Guanes

doi:10.2174/1874364102115010318

Abstract

Purpose: The aim of the study was to report the first case of a patient with Terrien’s Marginal Degeneration (TMD) who developed necrotizing anterior scleritis without systemic disease association, requiring systemic immunosuppressive treatment. Case Report: A 32-year-old female consulted for bilateral ocular burning and hyperemia. Initially, she was diagnosed with conjunctivitis and treated with topical antibiotics and corticosteroids, with mild transitory improvement but the progression of the disease. Years later, she attended the ocular immunology consultation for a second opinion where TMD with ocular inflammatory component OU was diagnosed. Seven months later, she presented with severe pain, decreased visual acuity, and photophobia in OS. At the slit-lamp examination, necrotizing anterior scleritis with a high risk of perforation in OS was observed. The patient was referred to the rheumatologist and started treatment with systemic corticosteroids and cyclophosphamide, exhibiting a clinical improvement. The patient did not meet the criteria for any systemic illness associated with scleritis, such as autoimmune diseases or vasculitis. Thus, scleritis was related to the adjacent inflammatory process associated with TMD, as an atypical presentation of this disease. Conclusion: Although an inflammatory type of TMD has been proposed, it is essential to follow up closely these patients and consider necrotizing anterior scleritis, a severe ocular disease that requires prompt immunosuppressive management, as a possible atypical associated presentation of this disease.

Highlights

Scleritis is a severe inflammatory condition characterized by edema and inflammatory cell infiltration of the sclera [1]
Terrien’s Marginal Degeneration (TMD) is a quite rare disease that usually presents in men between 40 and 59 years of age [3]
To the best of our knowledge, we present the first case of a woman with TMD who developed necrotizing anterior scleritis without systemic disease association, requiring systemic immunosuppressive treatment

Summary

Introduction

Scleritis is a severe inflammatory condition characterized by edema and inflammatory cell infiltration of the sclera [1]. Necrotizing anterior scleritis is divided into two forms, with inflammation or without inflammation (scleroma-lacia perforans). This disorder occurs more frequently in women older than 60 years and is frequently associated with systemic inflammatory diseases [2]. Terrien’s Marginal Degeneration (TMD) is a quite rare disease that usually presents in men between 40 and 59 years of age [3]. TMD typically presents as a unilaterally or asymmetrically bilateral peripheral corneal thinning, peripheral opacity with lipid deposition, corneal neovascularization, pseudopterygium formation, and astigmatism [3]

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