Successful treatment of Netherton's syndrome with topical calcipotriol.

We present a case of a 9-year-old boy with Netherton syndrome (NS) and skin manifestation of ichthyosis linearis circumflexa (ILC) who was successfully treated with topical 0.05% calcipotriol ointment bid. It was applied every fourth day on the same body area, which measured from 18% to 27% of the total body surface. Significant improvement of erythema and scaling was noted two weeks after the beginning of the treatment, with nearly total remission one week later, when the treatment was suspended. Remission lasted three to four weeks, when a few lesions of ILC appeared on his trunk and limbs and the treatment began again. The patient responded well each time he was treated. No adverse effects, suggestive of hypercalcemia or nephrocalcinosis, were noted during the treatment period which lasted for nine months. To evaluate calcipotriol's long-term efficacy and safety it should be tested on a larger group of patients with NS. Copyright John Libbey Eurotext 2003.