Lichen Myxedematosus: Atypical form and Therapeutic Success with Intravenous Immunoglobulin

Lichen myxedematosus is a cutaneous mucinosis of idiopathic origin. According to Rongioletti’s classification, 2006, lichen myxedematosus is classified into three forms. The scleromyxedema or lichen myxedematosus sclerodermiform and generalized is a form of lichen myxedematosus characterized by numerous papules and areas of cutaneous hardening due to the deposition of mucin in association with the increase of dermal collagen, in addition to monoclonal gammopathy and systemic manifestations that can be lethal. Another form of lichen myxedematosus is the localized one, distinguished by the presence of papules and cutaneous hardening in certain areas of the skin (sub classified into four subtypes: discrete papular form, acral persistent papular mucinosis, cutaneous mucinosis of infancy and pure nodular form). The third form is classified as atypical or intermediate and is characterized by clinical features of scleromyxedema but without monoclonal gammopathy, or also localized forms with monoclonal gammopathy and/or systemic symptoms [1-3].