[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.


METHODS
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.


CONCLUSIONS
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.


CONCLUSIONS
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.
Service de dermatologie, CHU La-Milétrie, avenue Jacques-Coeur, 86000 Poitiers, France. laetitia.lopez4@voila.fr
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Lopez
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Wierzbicka-Hainaut
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Villers
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Guillet
D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D007275:Injections, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D011701:PUVA Therapy; D053718:Scleromyxedema / Q000188:drug therapy* / Q000276:immunology*; D012712:Serum Globulins / Q000627:therapeutic use
D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D012712:Serum Globulins; D008775:Methylprednisolone