Combination Oral Prednisone and Intravenous Immunoglobulin in the Treatment of Scleromyxedema

Abstract

Background: Scleromyxedema is a clinical variant of the rare disease papular mucinosis that has both cutaneous and systemic manifestations. Treatment options are numerous and tend to be associated with serious potential side effects and frequent relapse. Objective: We report a case of scleromyxedema treated with low-dose oral prednisone and intravenous immunoglobulin (IVIg). This is followed by a review of the literature. Conclusion: IVIg is being used for a growing number of inflammatory and immune disorders. It is being increasingly reported as a successful treatment for scleromyxedema. Although our patient succumbed to the disease, combination therapy with prednisone and IVIg provided temporary symptomatic, laboratory, and clinical improvement of the condition. Optimization of this therapeutic strategy is thus indicated for the management of scleromyxedema.