Number patients | 2 | 3 |
Dose | 2 g/kg/5 days every month | 2 g/kg/5 days every month |
Side effects | None | None |
Variables considered | Only cutaneous improvement (skin scores) | Cutaneous and systemic improvement |
Results | In 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 months | In 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 |