Table 2

The result of our cases and those previously published are reported

Scleromyxoedema: response to high dose intravenous immunoglobulin (hdIVIg). J Am Acad Dermatol 2000;43:403–8Our cases
Number patients23
Dose2 g/kg/5 days every month2 g/kg/5 days every month
Side effectsNoneNone
Variables consideredOnly cutaneous improvement (skin scores)Cutaneous and systemic improvement
ResultsIn the first patient skin scores reduced over a three month period, but after one year his skin score returned to baselineIn the second patient skin scores reduced over three months and the patient's improvment was still maintained after 10 monthsIn the first and second cases, significant improvement of the skin thickening was seen, and in the third case the symptoms of encephalopathy also improved. In all cases, the beneficial results were long lasting