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Efficacy of intravenous immunoglobulin in Arndt-Gottron scleromyxedema.
Ann Dermatol Venereol. 2009 Apr;136(4):330-6.
[Article in French]
Lopez L, Wierzbicka-Hainaut E, Villers A, Guillet G.
Service de dermatologie, CHU La-Milétrie, avenue Jacques-Coeur, 86000 Poitiers, France.
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 (0.5g/kg per day for 3days) with a 2-month treatment
interval. DISCUSSION: IVIG at a dose of 2g/kg for 5days 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. 0.5g/kg per day for 3days. 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.
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