[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.
Lopez
L
L
Service de dermatologie, CHU La-Milétrie, avenue Jacques-Coeur, 86000 Poitiers, France. laetitia.lopez4@voila.fr
Wierzbicka-Hainaut
E
E
Villers
A
A
Guillet
G
G
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