[Efficacy of intravenous immunoglobulin in Arndt-Gottron scleromyxedema].

BACKGROUND: Arndt-Gottron scleromyxedema is characterized by cutaneous mucinosis occasionally associated with systemic disorders, and it has a chronic and unpredictable course. The lesions are due to abnormal fibroblastic activity leading to acid mucopolysaccharide deposits in tissue. We report the case of a 50-year-old woman treated with intravenous immunoglobulin (IVIG), with excellent results in terms of her cutaneous lesions.

CASE REPORT: A 50-year-old woman presented with multifocal micropapular lesions and cutaneous sclerosis that progressively limited joint movement and mouth opening and which were associated with a circulating monoclonal IgG Kappa. Previous treatments, including steroids, PUVA therapy, interferon, acitretin, methotrexate, melphalan and D-penicillamin were either ineffective or were stopped because of adverse effects. IVIG resulted in a remarkable and durable response at decreased dosage (0.5 g/kg per day for 3 days) with a 2-month treatment interval.

DISCUSSION: IVIG at a dose of 2g/kg for 5 days constitutes a valuable alternative treatment because of its immunomodulatory effect. In scleromyxedema without systemic lesions, we propose a maintenance regimen at lower dosage, i.e. 0.5 g/kg per day for 3 days.

CONCLUSION: Since Arndt-Gottron scleromyxedema is a rare disease, no standardized treatment has been yet established. The combination of minimal adverse effects and real efficacy makes IVIG an interesting alternative treatment for this disease.