Optimal Treatment Strategies for Chronic Cutaneous Vasculitis with Concurrent IGA Monoclonal Gammopathy: A Case Report

Abstract

Andres D. Parga, Elizabeth George and Donald Rudikoff

This case report investigates the complex presentation and management of a 61-year-old female patient with bilateral leg ulcerations, ultimately diagnosed with cutaneous vasculitis and concurrent IgA monoclonal gammopathy, classified as monoclonal gammopathy of clinical significance (MGCS). MGCS refers to nonmalignant monoclonal gammopathies that cause significant disease, often manifesting with symptoms affecting the nerves, kidneys, and skin. Our patient exhibited chronic ulcerations and a challenging clinical course over four years, requiring extensive investigation and multiple therapeutic interventions. Initial treatments, including high-dose corticosteroids and rituximab, provided minimal to limited improvement. However, significant clinical response was observed following the administration of hydroxychloroquine and dapsone. This case underscores the importance of recognizing MGCS in patients presenting with monoclonal gammopathy and unexplained symptoms, the critical role of skin biopsies in diagnosis, and the necessity of a multidisciplinary approach in managing these rare and complex conditions. The report highlights the complexities of diagnosing and treating cutaneous vasculitis associated with IgA monoclonal gammopathy and discusses the potential benefits of targeted therapies such as hydroxychloroquine and dapsone in achieving optimal outcomes.

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