AUTHOR=Wang Shang-shang , Chen Qin-yi , Xiang Lei-hong 

TITLE=Case report: Scleromyxedema associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins

JOURNAL=Frontiers in Immunology

VOLUME=Volume 13 - 2022

YEAR=2023

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2022.1099918

DOI=10.3389/fimmu.2022.1099918

ISSN=1664-3224

ABSTRACT=Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features are poorly documented. High-dose intravenous immunoglobulin has been suggested as the first-line treatment used either alone or in conjunction with systemic steroids and/or thalidomide. Herein we reported a 45-year-old woman diagnosed as scleromyxedema with paraproteinemia initially failed to respond to systemic steroids, retinoid and thalidomide but achieved great improvement after intravenous immunoglobulins treatment.