Scleromyxedema is a rare chronic skin mucinosis of unknown origin commonly associated with a monoclonal gammopathy of immunoglobulin G  type, defined by hematologists as monoclonal gammapathy of unknown significance. Mucinosis pathogenesis is currently considered to be a combination of indirect mechanisms like circulating cytokines, inflammatory mediators, and fibroblast precursor migration from the blood, their residence in dermis and in more extensive cases in other tissues and mucin synthesis. The new disease classification includes generalized lichen myxedematosus (scleromyxedema), localized lichen myxedematosus and atypical forms. This article deals with interdisciplinary approach to scleromyxedema work-up in terms of evaluation of patient with this disorder.