Validation of measurement scores for evaluating vascular anomaly skin lesions.

Vascular anomalies comprise a heterogeneous group of disorders caused by abnormal proliferation or development of vascular and/or lymphatic vessels. Vascular anomalies present with various symptoms and complications, but no standardized methods evaluate their severity, and to measure treatment outcomes is difficult. To assess the responsiveness of measurement scores for evaluating vascular anomaly skin lesions, we conducted a validation study to compare these measurement scores with patients' objective data. In this study, data were collected from treated and untreated patients. Skin lesions were photographed at baseline and after a follow-up period of 3-6 months. The volume of skin lesions, the degree of red or purple coloration, and color tone were measured objectively. Two external dermatologists evaluated patients' photographs and determined scores, which represented criteria for improvements in skin lesions (size and color) and 6-point Physician Global Assessment scores. The correlation between these scores and patients' objective data (lesion volume and color) was assessed to validate the scores. Twenty-three cases of vascular anomaly (seven vascular tumors, five lymphatic malformations, three venous malformations, and eight lymphatic-venous malformations) were examined. Scores for improvements in vascular anomaly skin lesions (size and color) correlated with a change in lesion volume, the degree of red or purple coloration, color tone score, and 6-point Physician Global Assessment score. Our findings suggest that these measurement scores are responsive to changes in vascular anomaly skin lesions after observation.

Intensive granulocyte and monocyte adsorption apheresis for generalized pustular psoriasis.

Granulocyte and monocyte adsorption apheresis (GMA) is usually performed weekly (consisting of five sessions) for refractory skin diseases, such as generalized pustular psoriasis (GPP). The time to remission of inflammatory bowel diseases has been reported to be significantly shorter in intensive GMA (twice a week) than in regular GMA (once a week). Despite several reports of GPP cases treated with intensive GMA, the efficacy of intensive GMA has not been verified in GPP. Herein, we present two GPP patients with a mutation in the IL36RN gene, who initially received regular GMA, and intensive GMA upon recurrence. There were no adverse effects during regular and intensive GMA for both patients. Because concomitant medication was only prednisolone (20 mg/day) during regular and intensive GMA, intensive GMA showed superiority to regular GMA in patient 1. Although concomitant medications were different between regular and intensive GMA in patient 2, these drugs had been used before the start of each GMA therapy. We cannot neglect the effects of concomitant drugs, but we observed a shorter time to remission in intensive GMA than that in regular GMA in both patients. More case studies will be necessary for evaluating the clinical efficacy of intensive GMA.

Genome-wide transcriptional orchestration of circadian rhythms in Drosophila.

Circadian rhythms govern the behavior, physiology, and metabolism of living organisms. Recent studies have revealed the role of several genes in the clock mechanism both in Drosophila and in mammals. To study how gene expression is globally regulated by the clock mechanism, we used a high density oligonucleotide probe array (GeneChip) to profile gene expression patterns in Drosophila under light-dark and constant dark conditions. We found 712 genes showing a daily fluctuation in mRNA levels under light-dark conditions, and among these the expression of 115 genes was still cycling in constant darkness, i.e. under free-running conditions. Unexpectedly the expression of a large number of genes cycled exclusively under constant darkness. We found that cycling in most of these genes was lost in the arrhythmic Clock (Clk) mutant under light-dark conditions. Expression of periodically regulated genes is coordinated locally on chromosomes where small clusters of genes are regulated jointly. Our findings reveal that many genes involved in diverse functions are under circadian control and reveal the complexity of circadian gene expression in Drosophila.