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

Abstract

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.

• Authors: L Lopez, E Wierzbicka-Hainaut, A Villers, G Guillet
• Journal: Annales de dermatologie et de venereologie (Ann Dermatol Venereol) Vol. 136 Issue 4 Pg. 330-6 (Apr 2009) ISSN: 0151-9638 [Print] France
• Vernacular Title: Efficacité des veinoglobulines dans le scléromyxoedème d'Arndt-Gottron.
• PMID: 19361699 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Immunoglobulins, Intravenous
• Immunologic Factors
• Serum Globulins
• Methylprednisolone

Topics

• Aged
• Aged, 80 and over
• Female
• Humans
• Immunoglobulins, Intravenous (administration & dosage, therapeutic use)
• Immunologic Factors (administration & dosage, therapeutic use)
• Injections, Intravenous
• Male
• Methylprednisolone (therapeutic use)
• Middle Aged
• PUVA Therapy
• Scleromyxedema (drug therapy, immunology)
• Serum Globulins (therapeutic use)