Mariko Tsujimoto

Limited influence of aspirin intake on mast cell activation in patients with food-dependent exercise-induced anaphylaxis: comparison using skin prick and histamine release tests.

Food-dependent exercise-induced anaphylaxis (FDEIA) is a severe systemic syndrome induced by physical exercise after ingesting causative food. Aspirin is a well-known trigger for anaphylaxis in patients with FDEIA. Possible mechanisms by which symptoms are aggravated by aspirin include enhanced antigen absorption and mast cell activation. The aim of this study was to determine whether aspirin intake has an influence on mast cell/basophil activation in patients with FDEIA. Provocation tests revealed that adding aspirin to the causative food challenge in 7 of 9 (77.8%) patients with FDEIA provoked symptoms. In most cases, pretreatment with aspirin did not enhance skin tests (71.4%) or histamine release tests (88.9%) with food allergen challenges. The study confirms that histamine release and skin prick tests can be adjunctive tools for diagnosing FDEIA. In addition, our results suggest that exacerbation of FDEIA symptoms by aspirin is not mediated by direct effects of aspirin on mast cell/basophil activation.

Steroid treatment can improve the impaired quality of life of patients with acquired idiopathic generalized anhidrosis

DEAR EDITOR, Acquired idiopathic generalized anhidrosis (AIGA) is characterized by systemic anhidrosis or hypohidrosis with no identified cause or other autonomic or neurological abnormalities. AIGA is sometimes accompanied by cholinergic urticaria with symptoms of tingling, unpleasant sensation and severe pruritus. Treatment options for AIGA are limited. Most reported cases responded to high-dose systemic corticosteroids including intravenous steroid pulse therapy. AIGA can lead to discomfort, hyperthermia and heat stroke, which may affect the quality of life (QoL) of patients. However, the relationship between the QoL of patients with AIGA and effectiveness of therapy has not been examined. The aim of this study was to examine the impact of AIGA on QoL and the relationship between therapeutic effectiveness and improvement in QoL. Eight male patients and one female patient with AIGA were enrolled in this study. The patient characteristics are described in Table 1. The diagnosis of anhidrosis and the treatment efficacy were assessed by a systemic thermoregulatory sweating test and local sweating test involving intradermal injection of acetylcholine and the starch–iodine test (Minor test). Other diseases with hypohidrosis such as Sj€ ogren syndrome and Fabry disease were excluded. Cholinergic urticaria was observed in seven patients. Histological findings revealed that morphological abnormalities of sweat glands and sweat ducts were absent in all patients, and slight infiltration of lymphocytes around the sweat glands was found in five patients. The mean interval between disease onset and treatment was 27 8 months. Histamine release test with the sweat or skin test using autologous sweat revealed sweat allergy in two of eight tested patients. One course of methylprednisolone pulse therapy (3 days, 1000 mg per day) was given to six patients, two courses were given to one patient and three courses were given to one patient. Sweating improved in all patients after therapy. Adverse effects and recurrence of symptoms were not observed for > 1 year after therapy. In the absence of therapy, anhidrosis remained unchanged in one patient who declined treatment. Skindex-16 inquires about the effect of skin conditions on QoL. Patients respond to questions by choosing one of seven possible response choices, which range from ‘never bothered’ to ‘always bothered’. Skindex responses are reported in three subscales (symptoms, emotions and functioning), which address three domains: symptoms, emotional effects, and effects on social and physical functioning. Scores vary from 0 (minimal effect on QoL) to 100 (significant effect on QoL). The mean Skindex-16 subscale scores before and after therapy are shown in Figure 1. Skindex-16 questions before therapy were carried out from spring to autumn (from May to November). Skindex-16 questions after therapy were performed in September (autumn in Japan). The average September temperature of our facility is 20–30 °C. Sweating tests were also performed alongside the Skindex-16 questionnaire before and after therapy.

Subcutaneous lobular capillary hemangioma with sonography and computed tomography findings

ejd.2012.1652 Auteur(s) : Ayuko Kikusawa1, Masahiro Oka1 oka@med.kobe-u.ac.jp, Hideki Shimizu1, Mariko Tsujimoto1, Yumiko Marui2, Shusaku Yokogawa3, Yasufumi Kita4, Makoto Kunisada1, Chikako Nishigori1 1 Division of Dermatology, Kobe University Graduate School of Medicine, Kobe 650-0017, Japan 2 Department of Clinical Laboratory, 3 Department of Radiology 4 Department of Surgery, Rokko Hospital, Kobe 657-0022, Japan Lobular capillary hemangioma (LCH), also known as pyogenic granuloma, is a common, [...]

Mitotic genes are transcriptionally upregulated in the fibroblast irradiated with very low doses of UV-C

Ultraviolet (UV) radiation induces a variety of biological effects, including DNA damage response and cell signaling pathways. We performed transcriptome analysis using microarray in human primary cultured fibroblasts irradiated with UV-C (0.5 or 5 J/m2) and harvested at 4 or 12 h following UV exposure. All transcript data were analyzed by comparison with the corresponding results in non-irradiated (control) cells. The number of genes with significantly altered expression (≥2-fold difference relative to the control) is higher in the sample irradiated with high dose of UV, suggesting that gene expression was UV dose-dependent. Pathway analysis on the upregulated genes at 12 h indicates that the expression of some cell cycle-related genes was predominantly induced irrespective of UV-dose. Interestingly, almost all the genes with significant altered expression were cell cycle-related genes designated as ‘Mitotic Genes’, which function in the spindle assembly checkpoint. Therefore, even a low dose of UV could affect the transcriptional profile.

Japanese case of xeroderma pigmentosum complementation group C with a novel mutation

Dear Editor, A 1-year-old boy was referred to Kobe University Hospital requesting a possible diagnosis of xeroderma pigmentosum (XP). He had had freckle-like pigmentation on his face since he was a baby. At 9 months, his mother took him to the hospital because the number of freckles had been increasing. Since then, sunlight had been he avoided, his parents applied sunscreen and he seldom spent time outdoors during the day. At presentation, he manifested many freckle-like pigmented maculae on his face and the back of his hands (Fig. 1a). He had never experienced exaggerated sunburn reactions such as blister formation or prolonged erythema upon minimum sun exposure. Skin cancer, mental retardation and neurological symptoms were not observed. His family did not have similar symptoms, and his parents were not consanguineous. A genetic complementation test was carried out by means of host-cell reactivation assay using fibroblasts derived from the patient. Luciferase activity was increased specifically when XPC cDNA was transfected into the patient’s cells, which suggested that the patient belonged to XP complementation group C (XP-C). Genome sequence analysis indicated that the patient harbored a compound heterozygous mutation of c.1024_1025insG and c.1950C>T, resulting in p.D342GfsX32 and p.R579X in XPC (Fig. 1b). Based on these findings, the patient was diagnosed as XP-C. We also confirmed the expression of XPC protein by western blot

A case of acute heart failure due to myocardial infiltration of mycosis fungoides

Mycosis fungoides (MF) has been reported to be the most common cutaneous lymphoma with a good prognosis and myocardial infiltration is clinically rare. We hereby report a case of rapidly progressing acute heart failure due to myocardial infiltration by MF. Perfusion cardiac magnetic resonance imaging (MRI) demonstrated a massive perfusion defect in the left ventricle (LV) where multiple nodular enhancement areas by delayed enhancement MRI could be documented in the postero-lateral wall of the LV, which resulted in a deterioration of the LV function and mitral regurgitation. Autopsy confirmed the myocardial infiltration by the MF, which corresponded with the MRI findings. <Learning objective: Symptomatic heart failure patients with myocardial infiltration by mycosis fungoides (MF) have a poor prognosis because they could not undergo chemotherapy for primary disease. Therefore, early diagnosis is important for improvement of prognosis. The routine assessment of the cardiac involvement by cardiac magnetic resonance imaging as well as transthoracic echocardiography should be performed for an early recognition of myocardial infiltration even in asymptomatic MF patients.>.

4-(4-Hydroxyphenyl)-2-butanol (rhododendrol)-induced melanocyte cytotoxicity is enhanced by UVB exposure through generation of oxidative stress

4‐(4‐Hydroxyphenyl)‐2‐butanol (rhododendrol, RD), a skin‐whitening agent, was reported to cause skin depigmentation in some users, which is attributed to its cytotoxicity to melanocyte. It was reported that cytotoxicity to melanocyte is possibly mediated by oxidative stress in a tyrosinase activity‐dependent manner. We examined the effect of UV radiation (UVR) on RD‐induced melanocyte cytotoxicity as an additional aggravating factor. UVR enhanced RD‐induced cytotoxicity in normal human epidermal melanocytes (NHEMs) via the induction of endoplasmic reticulum (ER) stress. Increased generation of intracellular reactive oxygen species (ROS) was detected. Pretreatment with N‐acetyl cysteine (NAC), antioxidant and precursor of glutathione significantly attenuated ER stress‐induced cytotoxicity in NHEMs treated with RD and UVR. Increase in cysteinyl‐RD‐catechol and RD‐pheomelanin in NHEMs treated with RD and UVR suggested that, after UVR excitation, RD or RD metabolites are potent ROS‐generating substances and that the tendency to produce RD‐pheomelanin during melanogenesis amplifies ROS generation in melanocytes. Our results help to elucidate the development mechanisms of RD‐induced leukoderma and provide information for innovation of safe skin‐whitening compounds.