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Scleromyxedema is a rare chronic fibromucinous disorder characterized by a generalized papular and sclerodermoid eruption. Despite its clinical significance, no definitive therapeutic guidelines exist for scleromyxedema, making management challenging. Herein, we present a case of a 76-year-old female patient referred for evaluation of systemic sclerosis, presenting with distinctive cutaneous manifestations and neurological symptoms.

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Scleromyxedema, a rare skin condition, is characterized by a waxy-appearing papular eruption that tends to impact middle-aged adults. Scleromyxedema is often linked to monoclonal gammopathies. However, some patients do not have a coinciding monoclonal gammopathy and experience an atypical presentation of the disease.

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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.

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Introduction: We provide a case report on a patient with scleromyxedema treated with high-dose intravenous immunoglobulins (IVIgs) who reported less fatigue and fewer headaches after transitioning to Yimmugo, a novel IVIg preparation. Scleromyxedema is a rare and chronic cutaneous mucinosis, frequently associated with monoclonal gammopathy and systemic complications, which may be life-threatening. The disease is characterized by papular eruptions and sclerosis of the skin.

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