Abstract: A 67-year-old male presented with papules and sclerosis all over the body for more than 4 years. Dermatological examination showed miliary-sized, waxy firm papules that partially coalesce into flakes, accompanied by firmness and loss of elasticity of the skin. Pathological showed that dermal fibroblast proliferation and mucin deposition. Laboratory examination showed IgG kappa monoclonal immunoglobulinemia, weakly positive ANA: 1∶100, slightly lower IgA: 0.88g/L. The diagnosis of scleromyxedema complicated with IgG κ paraproteinemia was made. The patient was treated with hormones and methotrexate for 2 months and the skin lesions improved significantly.

Key words: scleromyxedema, IgG κ monoclonal immunoglobulin