title: Scleromyxedema with subcutaneous nodules: successful treatment with thalidomide and intravenous immunoglobulin.
abstract: Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. In recent years, intravenous immunoglobulin (IVIg) appears to be the therapy of choice for scleromyxedema. Treatment experiences in atypical manifestations with mucinous nodules are limited to sporadic reports. We report the case of male patient with atypical scleromyxedema without underlying paraproteinemia, presenting with generalized papular and sclerodermoid skin eruption and multiple nodular mucinous lesions on the fingers and face as well as on the eyelids, and associated systemic symptoms. Complete regression of all cutaneous lesions and extracutaneous symptoms with sustained remission was achieved by combined treatment with thalidomide and IVIg.
authors.affiliation: Department of Dermatovenereology, University Medical Centre Ljubljana, Slovenia ; Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
authors.forename: M
authors.initials: M
authors.lastname: Dolenc-Voljč
authors.affiliation: Institute of Pathology, Faculty of Medicine, Ljubljana, Slovenia.
authors.forename: V
authors.initials: V
authors.lastname: Jurčić
authors.affiliation: Department of Rheumatology, University Medical Centre Ljubljana, Slovenia.
authors.forename: A
authors.initials: A
authors.lastname: Hočevar
authors.affiliation: Department of Rheumatology, University Medical Centre Ljubljana, Slovenia ; Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
authors.forename: M
authors.initials: M
authors.lastname: Tomšič
keywords: Intravenous immunoglobulin; Monoclonal gammopathy; Mucinosis; Nodular mucinosis; Papular mucinosis; Scleromyxedema; Thalidomide