Neurological disease in xeroderma pigmentosum. Documentation of a late onset type of the juvenile onset form.

Xeroderma pigmentosum (XP) is an autosomal recessive, neurocutaneous disorder characterized by sunlight-induced skin cancers and defective DNA repair. Many XP children develop a primary neuronal degeneration. We describe 2 unusual XP patients who had a delayed onset of XP neurological disease. Somatic cell genetic studies indicated that they have the same defective DNA repair gene and are both in XP complementation group A. These 2 patients, together with a group A patient previously reported from London, establish as a distinct clinical entity the late onset type of the juvenile onset form of XP neurological disease. The functional capacity of these patients' cultured fibroblast strains to survive after treatment with ultraviolet radiation indicates that their DNA repair defect is less severe than that of typical group A patients who have a more severe neurodegeneration with an earlier symptomatic onset. The premature death of nerve cells in XP patients (which is presumably due to their inherited defects in DNA repair mechanisms) suggests that normal repair of damaged DNA in neurons is required to maintain integrity of the human nervous system.

A new treatment for tinea versicolor using econazole nitrate 1.0 percent cream once a day.

Econazole nitrate 1.0 percent cream was compared with placebo for safety and efficacy in the treatment of 126 patients with tinea versicolor. Overall results showed that 97 percent of the patients who were treated with econazole nitrate 1.0 percent cream once a day achieved excellent or partial responses, while 62 percent of those patients applying the placebo cream attained similar responses. This difference is statistically significant (p = 0.001). The study results show that econazole nitrate cream is a valuable drug for the treatment of tinea versicolor.