Search

Background: Systemic sclerosis (SSc) is a multisystemic disease with an extensive microvasculopathy. Previously, disturbances in plasma levels of angiotensin II (Ang II) and its antagonistic angiotensin‐(1‐7) (Ang‐(1‐7)) were found in patients with SSc. Their significance in a pathogenesis of SSc stays unclear due to discrepancies of earlier studies. Objectives: To evaluate a significance of disturbances in production pathway of angiotensins in a development of SSc. Methods: There were enrolled 27 patients with established SSc, 23 subjects with very early SSc and 23 healthy controls. The diagnosis of SSc was established in patients who met EULAR/ACR 2013 classification criteria. Very early SSc described patients with Raynaud's phenomenon having SSc‐specific antinuclear antibodies and SSc‐like abnormalities in nailfold videocapillaroscopy. Patients were submitted to evaluation of internal organ involvement and blood sampling to assay plasma levels of angiotensin I, angiotensin II and angiotensin‐(1‐7) with ELISA technique. Results: Plasma level of angiotensin‐(1‐7) was significantly reduced in both SSc group (median = 47.2 pg/mL; P < 0.001) and ones with very early SSc (median = 102.7 pg/mL; P = 0.002) when compared to healthy controls (median = 176.1 pg/mL). A tendency to higher than in control group (median = 214 pg/mL) plasma level of angiotensin I was seen in SSc group (median = 392 pg/mL; P = 0.059). Differences in plasma level of angiotensin II were insignificant between all study groups. Those disturbances produced unfavourable angiotensin‐(1‐7)/angiotensin II (%) ratio in both groups of patients, which achieved statistical significance in subjects with established SSc (P < 0.001). Production pathway of angiotensins showed a dependence on a subtype of SSc, immune profile and a presence of interstitial lung disease. Conclusions: Production of angiotensin‐(1‐7) was significantly reduced in both SSc patients and those ones with very early SSc, although a significant imbalance between angiotensin II and angiotensin‐(1‐7) occurred only in subjects with established disease. [ABSTRACT FROM AUTHOR]

Introduction Psychological stress is known to cause exacerbation of different skin pathologies including alopecia areata ( AA). A hyperactivity of the hypothalamic-pituitary-adrenal axis (the HPA axis) in patients affected by AA ( AA patients) was proposed to be a neuroendocrine response to stress. Still little is known about melanocyte-stimulating hormone (MSH) and cortisol production in AA settings. Aim The aim of the study was to compare trends in a production of MSH and cortisol in patients with AA patients and healthy controls. Material and methods Plasma concentrations of free cortisol and MSH were measured in 43 AA patients (35.5 ± 10.6 years) and 37 healthy subjects (35.9 ± 10.5 years) selected from the Dermatology Outpatient Clinic at Medical University of Silesia in Katowice, Poland. Results were submitted to statistical analysis with Shapiro-Wilk W-test and subsequently nonparametric (Mann-Whitney U-test) or parametric (Student's t-test) statistics were performed. Results Mean plasma level of MSH was 5.39 ng/mL in AA patients and 5.71 ng/mL in healthy controls. The difference between groups was non-significant ( P = 0.435), but the control group manifested higher values of MSH (Q75 = 13.6 ng/mL vs Q75 = 5.98 ng/mL) and this tendency was especially stronger in females. AA patients had greater mean plasma level of cortisol (157.63 ± 91.16 µg/L) than healthy controls (123.32 ± 71.28 µg/L); however, the difference between them was also non-significant ( P = 0.063). No sex-dependent tendency to a greater production of cortisol was found. Conclusions Expectations of disturbances in production of MSH and cortisol were not fulfilled. Neither MSH nor cortisol plasma levels appear to be clearly changed in AA patients. [ABSTRACT FROM AUTHOR]

von Recklinghausen disease (vRD), more widely known as neurofibromatosis type 1, belongs to a group of genetic disorders and it is considered to be the most common genodermatosis. The disease has an autosomal dominant pattern of inheritance that involves mutations within the NF1 gene located on chromosome 17 in locus q11.2. The product of the NF1 gene is neurofibromin and the protein is well known to be a tumor suppressor factor. This counteracts possible overactivity of RAS (protein)/MAPK (mitogen-activated protein kinase) and RAS/PI3K/ AKT/ mTOR (phoshatydyloinositol-3-kinase/V-akt murine thy-moma viral oncogene homologue/mammalian target of rapamycin) signaling transduction pathways, preventing from uncontrolled cell proliferation and subsequent tumor formation. A loss of proper functioning of this protein leads to a development of vRD; however, a large variability in a phenotype of the disease and the onset of cutaneous findings, not necessarily in childhood, may provide a clinical diagnosis of the disease late in adulthood. We present a 52-year-old male in whom the diagnosis of vRD was proposed in the sixth decade of life, despite of multiple nodular lesions disseminated over the skin of the whole body and different neurological neurological disturbances, not considered for a long time as manifestations of genodermatosis. [ABSTRACT FROM AUTHOR]

von Recklinghausen disease (vRD), more widely known as neurofibromatosis type 1, belongs to a group of genetic disorders and it is considered to be the most common genodermatosis. The disease has an autosomal dominant pattern of inheritance that involves mutations within the NF1 gene located on chromosome 17 in locus q11.2. The product of the NF1 gene is neurofibromin and the protein is well known to be a tumor suppressor factor. This counteracts possible overactivity of RAS (protein)/MAPK (mitogen-activated protein kinase) and RAS/PI3K/AKT/mTOR (phoshatydyloinositol-3-kinase/V-akt murine thy-moma viral oncogene homologue/mammalian target of rapamycin) signaling transduction pathways, preventing from uncontrolled cell proliferation and subsequent tumor formation. A loss of proper functioning of this protein leads to a development of vRD; however, a large variability in a phenotype of the disease and the onset of cutaneous findings, not necessarily in childhood, may provide a clinical diagnosis of the disease late in adulthood. We present a 52-year-old male in whom the diagnosis of vRD was proposed in the sixth decade of life, despite of multiple nodular lesions disseminated over the skin of the whole body and different neurological disturbances, not considered for a long time as manifestations of genodermatosis. [ABSTRACT FROM AUTHOR]

Synopsis: Acne vulgaris is the most frequently diagnosed dermatosis in patients between 11 and 30. It is believed that it affects about 80% of persons in this age group or even, taking into account lesions of low intensity, 100% of young people. The aetiopathogenesis of acne is multifactorial. In all patients with acne, the following symptoms occur: excessive sebum production, excessive keratosis of excretory ducts and openings of sebaceous glands, development of bacterial flora and release of inflammatory mediators in the skin. Development of molecular biology, genetics and immunology contributed to a significant progress in studies on the pathogenesis of acne. Résumé: L'acne vulgaris est la dermatose la plus fréquemment diagnostiquée chez les patients entre 11 et 30 ans. On estime qu'il affecte environ 80% des personnes de ce groupe d'âge ou même, en tenant compte des lésions de faible intensité, 100% des jeunes. L'étiopathogénie de l'acné est multifactorielle. Chez tous les patients souffrant d'acné, les symptômes suivants se produisent: la production excessive de sébum, la kératose excessive des canaux excréteurs et les ouvertures de glandes sébacées, le développement de la flore bactérienne et la libération de médiateurs de l'inflammation de la peau. Le développement de la biologie moléculaire, la génétique et l'immunologie ont contribué à un progrès significatif dans les études sur la pathogenèse de l'acné. [ABSTRACT FROM AUTHOR]

Background: Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic sclerosis.Case Presentation: We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor.Conclusion: We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment.In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor. [ABSTRACT FROM AUTHOR]

Vitamin-A acetate is one of the most versatile vitamins. It is applied in medicine because of its antioxidative properties, in tumor therapy because of its cytostatic activity, and in cosmetics because of its nutritional additives. Herein, using broadband dielectric spectroscopy, the molecular dynamics of supercooled and glassy vitamin-A acetate was investigated. It was shown that dielectric measurements carried out at ambient and elevated pressures reveal a number of relaxation processes associated with different types of molecular motions: α, δ, and υ processes—observed above the glass transition temperature and the next two modes: β and γ identified in the glassy state. The occurrence of the δ mode in the dielectric spectrum may imply the existence of nanoscale domains with nematic order. This hypothesis is further checked by atomic force microscopy measurements. Finally, we have determined the value of the glass transition temperature (Tg) as well as the steepness index (mp) at various T-P conditions. [ABSTRACT FROM AUTHOR]