Author/Editor     Dolenc-Voljč, Mateja; Jurčić, Vesna; Hočevar, Alojzija; Tomšič, Matija
Title     Scleromyxedema with subcutaneous nodules
Type     članek
Vol. and No.     Letnik 5, št. 3
Publication year     2013
Volume     str. 309-315
ISSN     1662-6567 - Case reports in dermatology
Language     eng
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.
Keywords     scleromyxedema
intravenous immunoglobulin
Thalidomide
skleromiksedem
intravenski imunoglobulin
Thalidomide