Islam and socially responsible business conduct: An empirical research among Dutch entrepreneurs

This paper explores the relationship between the Islamic religion and the level of socially responsible business conduct (SRBC) of Islamic entrepreneurs. We find that the common idea of SRBC corresponds with the view of business in the Islam, although there are also some notable differences. We also find that Muslim entrepreneurs attach a higher weight to specific elements of SRBC than non- Muslims. But, on the other hand, we find that Muslims are less involved with applying SRBC in practice than non-Muslim managers. Furthermore, values and norms derived from the Islamic religion motivate entrepreneurs to contribute more to SRBC and lead to a higher commitment to specific aspects of SRBC compared to individually developed values and norms. Finally, the view of human nature of the Islam, preaching the natural goodness of man as a social being, leads to a positive view of SRBC

Islam and socially responsible business conduct: An empirical research among Dutch entrepreneurs

This paper explores the relationship between the Islamic religion and the level of socially responsible business conduct (SRBC) of Islamic entrepreneurs. We find that the common idea of SRBC corresponds with the view of business in the Islam, although there are also some notable differences. We also find that Muslim entrepreneurs attach a higher weight to specific elements of SRBC than non- Muslims. But, on the other hand, we find that Muslims are less involved with applying SRBC in practice than non-Muslim managers. Furthermore, values and norms derived from the Islamic religion motivate entrepreneurs to contribute more to SRBC and lead to a higher commitment to specific aspects of SRBC compared to individually developed values and norms. Finally, the view of human nature of the Islam, preaching the natural goodness of man as a social being, leads to a positive view of SRBC.Business conduct; Dutch entrepreneurs

Islam and socially responsible business conduct: An empirical research among Dutch entrepreneurs

This paper explores the relationship between the Islamic religion and the level of socially responsible business conduct (SRBC) of Islamic entrepreneurs. We find that the common idea of SRBC corresponds with the view of business in the Islam, although there are also some notable differences. We also find that Muslim entrepreneurs attach a higher weight to specific elements of SRBC than non- Muslims. But, on the other hand, we find that Muslims are less involved with applying SRBC in practice than non-Muslim managers. Furthermore, values and norms derived from the Islamic religion motivate entrepreneurs to contribute more to SRBC and lead to a higher commitment to specific aspects of SRBC compared to individually developed values and norms. Finally, the view of human nature of the Islam, preaching the natural goodness of man as a social being, leads to a positive view of SRBC

Epidermolysis bullosa simplex generalized severe induces a T helper 17 response and is improved by apremilast treatment

International audienceBACKGROUND: Epidermolysis bullosa simplex generalized severe is a genetic disorder caused by mutation in KRT5 or KRT14 genes. Usually considered as a mechanical disease, recent data argue for additional inflammatory mechanisms. OBJECTIVES: The aim of this study was to assess the inflammation in the skin of patients with EBS. METHODS: A first immunohistochemical retrospective study was performed on frozen skin samples from 17 EBS-gen sev patients. A second multicenter prospective study was conducted on 10 patients with severe EBS-gen sev. Blister fluid and epidermis were processed for immunochemistry analysis and quantitative real time PCR. Cytokine expression was analyzed in blister fluid and compared with controls. RESULTS: Histological analysis showed a constant dermal perivascular CD4+ lymphocytes infiltrate in skin biopsies of blister (n=17) as well as in rubbed skin (n=5), an epidermal infiltration of neutrophils and eosinophils in 70% of cases and an increased immunostaining for CXCL9 and CXCL10 in blistering skin. High levels of Th17 cytokines were detected in lesional skin. Three adult patients with EBS-gen sev were treated with apremilast with a dramatic improvement of skin blistering and good tolerance. CONCLUSION: Our study demonstrates the importance of inflammation in EBS-gen sev patients and underlines the key role for Th17 cells in its pathogenesis. In addition, this study provides promising new therapeutic approaches for this disabling disorder. This article is protected by copyright. All rights reserved

Proposal for a 6-step approach for differential diagnosis of neonatal erythroderma

The broad differential diagnosis of neonatal erythroderma often poses a diagnostic challenge. Mortality of neonatal erythroderma is high due to complications of the erythroderma itself and the occasionally severe and life-threatening underlying disease. Early correct recognition of the underlying cause leads to better treatment and prognosis. Currently, neonatal erythroderma is approached on a case-by-case basis. The purpose of this scoping review was to develop a diagnostic approach in neonatal erythroderma. After a systematic literature search in Embase (January 1990 - May 2020, 74 cases of neonatal erythroderma were identified, and 50+ diagnoses could be extracted. Main causes were the ichthyoses (40%) and primary immunodeficiencies (35%). Congenital erythroderma was present in 64% (47/74) of the cases, predominantly with congenital ichthyosis (11/11; 100%), Netherton syndrome (12/14, 86%) and Omenn syndrome (11/23, 48%). Time until diagnosis ranged from 102 days to 116 days for cases of non-congenital erythroderma and congenital erythroderma respectively. Among the 74 identified cases a total of 17 patients (23%) died within a mean of 158 days and were related to Omenn syndrome (35%), graft-versus-host disease (67%) and Netherton syndrome (18%). Disease history and physical examination are summarized in this paper. Age of onset and a collodion membrane can help to narrow the differential diagnoses. Investigations of blood, histology, hair analysis, genetic analysis and clinical imaging are summarized and discussed. A standard blood investigation is proposed, and the need for skin biopsies with lympho-epithelial Kazal-type related Inhibitor staining is highlighted. Overall, this review shows that diagnostic procedures narrow the differential diagnosis in neonatal erythroderma. A 6-step flowchart for the diagnostic approach for neonatal erythroderma during the first month of life is proposed. The approach was made with the support of expert leaders from international multidisciplinary collaborations in the European Reference Network Skin-subthematic group Ichthyosis.Peer reviewe

The Sanriku Project

FSW – Publicaties zonder aanstelling Universiteit Leide

Clinical spectrum of MTOR-related hypomelanosis of Ito with neurodevelopmental abnormalities

PURPOSE: Hypomelanosis of Ito (HI) is a skin marker of somatic mosaicism. Mosaic MTOR pathogenic variants have been reported in HI with brain overgrowth. We sought to delineate further the pigmentary skin phenotype and clinical spectrum of neurodevelopmental manifestations of MTOR-related HI. METHODS: From two cohorts totaling 71 patients with pigmentary mosaicism, we identified 14 patients with Blaschko-linear and one with flag-like pigmentation abnormalities, psychomotor impairment or seizures, and a postzygotic MTOR variant in skin. Patient records, including brain magnetic resonance image (MRI) were reviewed. Immunostaining (n = 3) for melanocyte markers and ultrastructural studies (n = 2) were performed on skin biopsies. RESULTS: MTOR variants were present in skin, but absent from blood in half of cases. In a patient (p.[Glu2419Lys] variant), phosphorylation of p70S6K was constitutively increased. In hypopigmented skin of two patients, we found a decrease in stage 4 melanosomes in melanocytes and keratinocytes. Most patients (80%) had macrocephaly or (hemi)megalencephaly on MRI. CONCLUSION: MTOR-related HI is a recognizable neurocutaneous phenotype of patterned dyspigmentation, epilepsy, intellectual deficiency, and brain overgrowth, and a distinct subtype of hypomelanosis related to somatic mosaicism. Hypopigmentation may be due to a defect in melanogenesis, through mTORC1 activation, similar to hypochromic patches in tuberous sclerosis complex