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Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms

ORPHA:90399· ICD-10 L98.5

Definition

Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms is a form of atypical lichen myxedematosus, characterized by the appearance of several 2-4 mm erythematous waxy papules confined to a few sites that may be associated with either an immunoglobulin A (IgA) nephropathy in patients with acral persistent papular mucinosis; discrete papular lichen myxedematosus; a scleromyxedema-like involvement, with dysphagia, hoarseness, pulmonary involvement, and carpal tunnel syndrome; myositis without skin sclerosis; or paraproteinemia.

Prevalence
<1 / 1 000 000
Age of onset
Adult, Elderly