Generalized pustular psoriasis (GPP) is a rare disease characterized by recurrent fever and systemic flushing accompanied by extensive sterile pustules. The committee of the guidelines was founded as a collaborative project between the Japanese Dermatological Association and the Study Group for Rare Intractable Skin Diseases under the Ministry of Health, Labour, and Welfare Research Project on Overcoming Intractable Diseases. The aim of the guidelines was to provide current information to aid in the treatment of patients with GPP in Japan. Its contents include the diagnostic and severity classification criteria for GPP, its pathogenesis, and recommendations for the treatment of GPP. Since there are few clinical trial data with high levels of evidence for this rare disease, recommendations by the committee are described in the present guidelines.

BACKGROUND: A self-administered questionnaire on dietary habits used in the JACC Study contained a 40-item food frequency questionnaire (FFQ). Although more than 110 thousand subjects enrolled in JACC Study and responded to the FFQ, no validation study has been conducted to date. METHODS: Eighty-five volunteers among the cohort members completed 2 FFQs (FFQs 1&2) and 12-day weighed dietary records (WDR). The interval between the two FFQs was one year. During the one year, the subjects carried out a 3-consecutive-day WDR in each season. We tested the reproducibility by using two FFQs. Also, we tested the validity of the FFQ by using the 12-day WDR as a gold standard. RESULTS: The intake frequencies of the 2 FFQs often agreed, showing the Spearman correlation coefficients ranging from 0.42 (edible wild plants) to 0.86 (coffee). The Spearman correlation coefficients of the energy and nutrient intakes from FFQ2, and that of the 12-day WDR were 0.20(energy) to 0.46 (animal protein, potassium). After adjusting the energy intake, the correlation coefficients showed 0.21(fish fat) to 0.51(animal fat). When classifying the FFQ2 and WDR by quartiles and examining the degree of agreement between the two methods, we obtained its median 30%. CONCLUSIONS: The FFQ is suitable to deal with a large group of subjects. However, since the energy and the amount of nutrient intake from this FFQ can not show the overall dietary intake situation, the subjects’ dietary intake should be assessed by categories.

In the current study, smoking was negatively associated with H. pylori infection. The risk of H. pylori seropositivity decreased linearly with cigarette consumption per day. Increased gastric acidity in the stomach through smoking may be a cause of the dose-dependently negative association between H. pylori and smoking. Drinking was negatively and dose-dependently associated with H. pylori positivity, although the effect of drinking was weaker than that of smoking.

Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS) is a severe adverse drug reaction caused by specific drug. It is characterized by visceral organ involvement and reactivation of various human herpesviruses. Although sporadic reports have documented certain conditions that appear after the resolution of DIHS/DRESS, little information is available on sequelae after resolution of DIHS/ DRESS in a large patient population. The Asian Research Committee on Severe Cutaneous Adverse Reactions, comprised of doctors from Japan and Taiwan, conducted a survey on sequelae and deterioration of the underlying disease in patients with DIHS/DRESS. This was achieved by directly interviewing patients who had been followed-up by experts or through a questionnaire mailed to patients. Questions were asked about new onset cardiovascular disease, collagen disease or autoimmune disease, gastrointestinal disease, renal disease, respiratory disease, neoplasms, and other diseases such as herpes zoster and diabetes mellitus, as well as deterioration of the underlying disease. A total of 145 patients were analyzed in this study. The following newly developed diseases after recovery from DIHS/DRESS were observed: Graves' disease (n = 2), Hashimoto's disease (n = 3), painless thyroiditis (n = 2), fulminant type 1 diabetes mellitus (n = 5), and infectious diseases (n = 7). Several DIHS/ DRESS patients with pre-existing renal dysfunction required lifelong hemodialysis. DIHS/DRESS is a condition that increases the risk of new onset of disease. Long-term observation of DIHS/DRESS can provide an opportunity to investigate substantial diseases from onset to the full-blown stage. Patients with DIHS/DRESS require careful long-term follow-up.

The Committee for Guidelines for the Management of Pemphigus was organized as one element of the Japanese Dermatological Association (JDA) and the Ministry of Health, Labour, and Welfare (MHLW) Research Project on Measures for Research Committee for Intractable Skin Disease. Pemphigus has been defined as a group of intractable autoimmune blistering diseases caused by anti-desmoglein 1 and/or anti-desmoglein 3 IgG autoantibodies by the MHLW. The diagnosis of this condition and the criteria for assessing its severity are based on suggestions from the MHLW Research Group. The clinical practice guidelines presented here are those that are currently recommended in Japan. However, symptoms and complications can vary widely among individual pemphigus patients, so not all therapies will be required to be in complete agreement with these guidelines.