Severe cutaneous scleromyxedema treated by lenalidomide

Scleromyxedema is a rare clinical variant of primary cutaneous mucinosis. It defined by generalized papular and sclerodermoid eruption associated with monoclonal gammopathy. No specific definitive treatment known those tried have inconsistent results. The first-line intravenous immunoglobulin infusions variable response frequent relapse. We describe, here, patient scleromyxedema lambda gammopathy treated effectively low-dose lenalidomide dexamethasone. There was no or biological relapse after 3 years follow-up. In our opinion, agents targeting the abnormal plasma cell clone like are most promising in scleromyxedema.