Therapeutic Plasma Exchange for the Symptomatic Treatment of Scleromyxedema: A Case Report and Literature Review

Abstract

Scleromyxedema is a rare skin mucinosis often associated with systemic involvement and monoclonal gammopathy (MG). No formal recommendation for management with therapeutic plasma exchange (TPE) has been published due to rarity. This paper reports a 42-year-old male with progressive scleromyxedema. The patient was treated with glucocorticoids, immunosuppressants, and intravenous immunoglobulin (IVIG) therapy, but the disease progressed. Ultimately, the patient was referred for TPE, which resulted in decreased skin stiffness, improved extremity range of motion, and decreased visibility of papules on the hands. The patient's debilitating dysphagia gradually improved. A review of the literature focusing on clinical response identified five cases of scleromyxedema patients treated with TPE. Three of the five cases reported significant improvement in cutaneous symptoms and range of motion for at least 12 months. Overall, we propose that TPE should be considered an effective supportive treatment for symptomatic relief in severe or refractory scleromyxedema.