Sarcoidosis is a multisystem granulomatous disease of unknown aetiology with variable manifestations, which may affect virtually any organ. Muscular sarcoidosis is a rare entity, and among this group of muscular lesions, the tumour-like muscular sarcoidosis subtype is extremely rare. We report on two sarcoidosis cases that presented muscular sarcoid lesions with subcutaneous tumours.
Pollen-food allergy syndrome (PFAS) is a relatively rare form of food allergy which develops in individuals who are sensitized to pollen. Tree pollens, especially birch pollen, frequently induce PFAS; however, the incidence of PFAS due to grass or weed pollens such as ragweed or mugwort is relatively rare. Mugwort-mustard allergy syndrome (MMAS) is an example of a PFAS in which individuals sensitized to mugwort may develop an allergy to mustard and experience severe reactions. We herein describe a case of MMAS due to broccoli consumption.
Abstract Hailey‐Hailey disease (HHD) is an autosomal dominant genetic disease caused by a mutation of the ATP2C1 gene. Corticosteroids, antibiotics or cyclosporine have been administered to reduce inflammation and prevent flare‐ups, but the efficacy is not always sufficient. We herein report two cases of HHD effectively treated with apremilast and review the previous literature. Patient 1 was a 28‐year‐old male and patient 2 was a 35‐year‐old female. Both patients were diagnosed with HHD based on histological and genetic analyses. Both patients were treated with oral antibiotics or topical corticosteroids, but their symptoms were refractory, therefore apremilast was administered to both patients. Two weeks later, the skin lesion of both patients was improved. No adverse reaction was observed except for mild headache in patient 2. There have been 13 reported cases of HHD treated with apremilast, including our cases. Eight cases showed a good response to apremilast, whereas five cases showed no response. There seems to be no association between the disease severity and efficacy of apremilast, although the reason remains unknown. Interestingly, an early improvement of the HHD lesion was observed in all good response cases. Although digestive symptoms, headache, and myalgia were observed as adverse events, the treatment was well‐tolerated. The accumulation of a greater number of similar cases and further research will be required. We hypothesize that apremilast may be a useful therapeutic option for skin lesions of HHD.
Journal Article Conversion from human haematopoietic stem cells to keratinocytes requires keratinocyte secretory factors Get access Y. Fujita, Y. Fujita Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Dr Yasuyuki Fujita, Department of Dermatology, Hokkaido University Graduate School of Medicine, N15 W7, Kita‐ku, Sapporo 060‐8638, Japan E‐mail: yfujita@med.hokudai.ac.jp Search for other works by this author on: Oxford Academic Google Scholar D. Inokuma, D. Inokuma Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Search for other works by this author on: Oxford Academic Google Scholar R. Abe, R. Abe Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Search for other works by this author on: Oxford Academic Google Scholar M. Sasaki, M. Sasaki Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Search for other works by this author on: Oxford Academic Google Scholar H. Nakamura, H. Nakamura Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Search for other works by this author on: Oxford Academic Google Scholar T. Shimizu, T. Shimizu Department of Dermatology, Faculty of Medicine, University of Toyama, Toyama, Japan Search for other works by this author on: Oxford Academic Google Scholar H. Shimizu H. Shimizu Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan Search for other works by this author on: Oxford Academic Google Scholar Clinical and Experimental Dermatology, Volume 37, Issue 6, 1 August 2012, Pages 658–664, https://doi.org/10.1111/j.1365-2230.2011.04312.x Published: 01 August 2012
We investigated the involvement of serine protease and proteinase-activated receptor 2 (PAR2) in dermatophyte-induced itch in mice. An intradermal injection of an extract of the dermatophyte Arthroderma vanbreuseghemii (ADV) induced hind-paw scratching, an itch-related behavior. ADV extract-induced scratching was inhibited by the opioid receptor antagonists naloxone and naltrexone, the serine protease inhibitor nafamostat mesylate, and the PAR2 receptor antagonist FSLLRY-NH2. ADV extract-induced scratching was not inhibited by the H1 histamine receptor antagonist terfenadine or by mast cell deficiency. Heat pretreatment of the ADV extract markedly reduced the scratch-inducing and serine protease activities. Proteolytic cleavage within the extracellular N terminus of the PAR2 receptor exposes a sequence that serves as a tethered ligand for the receptor. The ADV extract as well as tryptase and trypsin cleaved a synthetic N-terminal peptide of the PAR2 receptor. The present results suggest that serine protease secreted by dermatophytes causes itching through activation of the PAR2 receptors, which may be a causal mechanism of dernatophytosis itch.
X-linked dyskeratosis congenita (DC) is an inherited disease caused by mutations in the DKC1 gene[1]. In typical cases, abnormal skin pigmentation and nail changes usually appear first, often by 10 years of age, with bone marrow failure and death developing before 20 and 40 years of age, respectively [2]. We report a patient with X-linked DC with a late presentation, where a substitution of methionine to threonine at position 350 in the dyskerin protein was found.A 35-year-old Japanese male presented [...]
Objective: Coronary microcirculation plays an important role in the progression of cardiac remodeling. Among angiogenic factors, it has been reported that angiotensin II may contribute to neovascularization. However, it is unknown whether inhibition of the renin–angiotensin system suppresses angiogenesis, especially within the heart. Our aim was to evaluate the effects of the angiotensin-converting enzyme inhibitor enalapril and the angiotensin II receptor type I blocker valsartan on cardiac microvasculature, function, vascular endothelial growth factor (VEGF) expression, and survival in cardiomyopathic hamsters. Methods: Male cardiomyopathic hamsters (BIO TO2) were administered either a placebo (group C), enalapril (30 mg/kg/day) (group E), or valsartan (40 mg/kg/day) (group V), starting at the age of 6 weeks. This continued until death. Hemodynamic study, histological analysis, and northern blot analysis were performed at 39 weeks. Results: Group V showed significant increases in percent fibrosis, end diastolic pressure, and LV dP/dt min, and significant decreases in percent fractional shortening, LV dP/dt max, capillary density, and the level of mRNA expression of VEGF compared with group C. Group E showed significant increases in percent fractional shortening while the capillary density and level of mRNA expression of VEGF were unchanged. The 300-day survival rate was significantly lower in group V (25.0%) but higher in group E (100%) than that of group C (66.7%). Conclusions: Therapy with valsartan may have adverse effects on survival rate concomitant with the progression of cardiac remodeling owing to impaired VEGF-mediated angiogenesis. Therapy with enalapril has a neutral effect on VEGF-mediated angiogenesis, leading to the suppression of cardiac remodeling and an increase in life expectancy.
Abstract: We have previously shown that human epidermal keratinocytes express macrophage migration inhibitory factor (MIF) mRNA, and immunohistochemical studies showed that MIF is expressed in human epidermis. To explore the possible pathophysiological roles of MIF in skin during rat fetal development, we examined the expression patterns of MIF during rat epidermal development using Northern blot analysis and in situ hybridization. Expression of MIF mRNA was first detected by in situ hybridization in the developing epidermis and hair germ cells from embryonic day (ED) 16. From ED 19, moderate levels of MIF expression were detected in the epidermis and epithelial sheath cells of growing hair follicles. In postnatal rat skin, higher MIF expression was detected in the epidermis and hair follicles on postnatal day 3. These observations were also confirmed by Northern blot analysis. Immunohistochemical analysis with an anti‐MIF antibody showed a similar distribution to that of the mRNA. Our results suggest that MIF is associated with epidermal and hair follicle development.
Background Vitiligo is an acquired pigmentary disorder characterized by depigmented patches on the skin that majorly impact patients' quality of life. Although its etiology involves genetic and environmental factors, the role of microorganisms as environmental factors in vitiligo pathology remains under-researched. Objectives Our study explored the presence of characteristic bacterial and fungal flora in vitiligo-affected skin and investigated their potential roles in vitiligo pathogenesis. Methods We sequenced bacterial 16 S rRNA and the fungal ITS1 region from skin swabs collected at frequently affected sites, namely the forehead and back, of patients with vitiligo. We analyzed bacterial and fungal flora in lesional and non-lesional areas of patients with vitiligo compared with corresponding sites in age- and sex-matched healthy subjects. Results Our findings revealed elevated α-diversity in both bacterial and fungal flora within vitiligo lesions compared with healthy controls. Notably, bacterial flora exhibited a distinctive composition in patients with vitiligo, and the proportional representation of Enterococcus was inversely correlated with the degree of vitiligo progression. Gammaproteobacteria, Staphylococcus spp., and Corynebacterium spp. were more abundant in vitiligo patients, with notable Staphylococcus spp. prevalence during the stable phase on the forehead. Conversely, the proportion of Malassezia sympodialis was lower and that of Malassezia globosa was higher in the progressive phase on the back of vitiligo patients. Conclusion Our study identified some characteristic bacterial and fungal groups associated with vitiligo activity and prognosis, highlighting the potential roles of microorganisms in pathogenesis and offering insights into personalized disease-management approaches.
