Objective. To assess the efficacy and tolerability of hydroxychloroquine (HCQ) in patients with cutaneous lupus erythematosus (CLE), in a phase III clinical trial conducted in Japan.Methods. We conducted a double-blind, randomized, parallel-group clinical trial. This was a baselinecontrolled study, and the group differences were evaluated in an exploratory analysis. A total of 103 patients with active CLE (according to a Cutaneous Lupus Erythematosus Disease Area and Severity Index [CLASI] activity score of ‡4) were included. Patients were randomized 3:1 to receive HCQ or placebo during the 16-week doubleblind period, and all patients were given HCQ during the following 36-week single-blind period. The primary efficacy end point was a reduction in the CLASI activity score at week 16. The secondary end points included the central photo evaluation (5-point scale), patient's global assessment (7-point scale), the Skindex-29 score, and investigator's global assessment (7-point scale, based on the other 3 secondary end points). In patients with systemic lupus erythematosus, fatigue and musculoskeletal pain were assessed. Safety was assessed up to week 55.Results. The mean CLASI score at week 16 was significantly improved from baseline in both the HCQ group and the placebo group: mean change 24.6 (95% confidence interval [95% CI] 26.1, 23.1) (P < 0.0001), and mean change 23.2 (95% CI 25.1, 21.3) (P 5 0.002), respectively, without between-group difference (P 5 0.197). The investigator's global assessment demonstrated a greater proportion of "improved" and "remarkably improved" patients in the HCQ group (51.4% versus 8.7% in the placebo group [P 5 0.0002 between groups]). The other secondary end points supported the efficacy of HCQ. Cellulitis, drug eruption, hepatic dysfunction, and Stevens-Johnson syndrome ClinicalTrials.gov identifier: NCT01551069. Supported

Polyarteritis nodosa (PN) is a classical collagen disease with poor prognosis that demonstrates systemic necrotizing vasculitis of small and medium-sized arteries. Cutaneous symptoms are observed in 25-60% of PN patients. On other hand, cutaneous polyarteritis nodosa (CPN) is designated for the cutaneous limited form of PN and demonstrates benign prognosis. However, there has been much debate on whether or not CPN can progress to PN. Although CPN lesions are fundamentally limited to skin, some CPN cases show extracutaneous symptoms such as peripheral neuropathy and myalgia. According to PN diagnostic criteria, which were established by the Ministry of Health, Labour and Welfare of Japan, a disease with both cutaneous and at least one extracutaneous symptom with appropriate histopathological findings can be diagnosed as PN. The same is true according to diagnostic criteria established by the American College of Rheumatology. In addition, there are no specific diagnostic criteria for CPN. In this study, CPN cases were retrospectively collected from multiple Japanese clinics, and analyzed for detailed clinical and histopathological manifestations, in order to redefine the clinical entity of CPN and to propose appropriate diagnostic criteria for CPN and PN. According to the CPN description in Rook's Textbook of Dermatology, we collected 22 cases with appropriate histopathological findings. Of the 22 cases, none progressed to PN or death during the follow-up period, 32% had peripheral neuropathy and 27% had myalgia. Regarding extracutaneous symptoms with CPN, 17 dermatological specialists in vasculitis sustained the opinion that CPN can be accompanied by peripheral neuropathy and myalgia but these symptoms are limited to the same area as skin lesions. Based on these results, we devised new drafts for CPN and PN diagnostic criteria. Our study shows the efficacy of these criteria and most dermatologists recognized that our new diagnostic criteria for CPN and PN are appropriate at the present time. In conclusion, this study suggests that CPN does not progress to PN, and introduces new drafts for CPN and PN diagnostic criteria.

BackgroundIn Ghana’s health delivery services, facilitative supervisory visit (FSV) as a system of management is new. This paper presents the standard evaluation results of FSV, which formed an integral part of the community-based health planning services (CHPS) initiative.MethodsThe study was conducted in the Upper West Region of Ghana. The Project developed guidelines and tools for FSV for four different health system levels – regional, district, sub-district and community levels. Electronic data from all four levels representing quarterly results were compiled into their annual equivalents, and summarized graphically for comparison.ResultsThe data show that all the nine districts embraced the FSV concept even though they differed markedly with regard to the degree of adherence to some set benchmarks. Three DHMTs (Wa Municipal, Lawra and Jirapa) were graded as good while the remaining six DHMTs were adjudged as fair in relation to management of supplies, transport and equipment, information, meeting, and technical support.ConclusionsThe data further suggest that there is much to gain both individually and institutionally from FSVs. Generally, FSVs are crucial to the delivery of primary health care services in especially rural areas.

The quality of life (QOL) of lupus erythematosus (LE) patients with skin manifestations is impaired, but little is known about Japanese patients. We assessed whether the skin symptoms in LE are associated with the QOL using the Japanese versions of the Skindex-29 and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). In all, 54 LE patients with cutaneous lesions completed the Japanese version of the Skindex-29, and physicians assessed the severity of their eruptions using the CLASI before and after treatment. The QOL of the LE patients was better after the therapeutic intervention using the Skindex-29 questionnaire. We tested several factors for an independent association with the QOL. A significant risk factor for a poor QOL was a female gender in "Functioning" before treatment. In addition, a poor QOL tended to be correlated with a female gender in "Emotions" and older current age in "Symptoms" before treatment, and with a longer duration of SLE in "Functioning" after treatment. In the CLASI analysis, skin manifestation activity in the acute phase correlated with a poor emotional and functional QOL rather than a symptomatic QOL. To the best of our knowledge, this is the first report evaluating the QOL of Japanese LE patients, despite the small cohort.

Lupus erythematosus (LE) includes a broad spectrum of diseases from a cutaneous-limited type to a systemic type. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects multiple organs. Cutaneous lupus erythematosus (CLE) includes skin symptoms seen in SLE and cutaneous-limited LE. Although immune abnormalities, as well as heritable, hormonal and environmental factors, are involved in the pathology of LE, the actual pathogenesis is still unclear. Recently, the involvement of various cytokines has been shown in the pathogenesis of LE. Moreover, some trials with biological agents targeted specific cytokines are also ongoing for SLE. In this article, we review the contributions of major cytokines such as interferon, tumor necrosis factor-α and interleukin-18 to LE, especially SLE and CLE.