Intravenous Immunoglobulins: an eye opener on the successful treatment of severe adult-onset paraprotein-associated xanthogranulomatosis.

Xanthogranulomatosis (XG) is a granulomatous dermatitis defined as multiple xanthogranulomas. XG is a common non-Langerhans histiocytosis mainly observed in children (juvenile XG) and usually regresses over time. It was first described in adults in 1963 and consists of persistent lesions.1 The most frequent associations are hematologic disorders including monoclonal gammopathies of undetermined significance (MGUS).2,3 To date, there are no guidelines for the treatment of XG. We hereby report two cases of XG successfully treated with intravenous immunoglobulins (IVIG).