Evaluation of neurodevelopmental symptoms in 10 cases of neonatal ichthyosis and sclerosing cholangitis syndrome.

Neonatal ichthyosis and sclerosing cholangitis (NISCH) syndrome is an extremely rare entity with only 19 patients described in the literature. We report an extended family with the disorder and investigate the association of neurodevelopmental symptoms. Patients with CLDN1 mutations, and specifically « the Moroccan¼ c.200_201delTT deletion, may be an increased risk for neurodevelopmental symptoms such as learning disabilities, mental retardation, and language delay.

[A new vision of skin]. / Une nouvelle vision de la peau.

As of today, the skin will never look the same. Indeed, several technologies allowing the observation of the skin at a microscopic level are now coming of age. This magnifies our visual acuity and thus our capacity to recognize various pathological processes. We herein present three different methods of non-invasive skin imaging we have worked with at the CHUV, which revolutionize the practice of dermatology. In vivo confocal microscopy, ex-vivo confocal microscopy and optical coherence tomography are complementary techniques that are now becoming essential for the modern management of skin cancers, among other uses.

Dès aujourd'hui, vous ne verrez plus la peau de la même manière. En effet, plusieurs technologies de microscopie sur peau intacte arrivent à maturité. Ceci décuple notre acuité visuelle, et donc notre capacité à reconnaître différents processus pathologiques. Nous présentons ici trois techniques d'imagerie cutanée non invasive avec lesquelles nous avons travaillé au CHUV, et qui sont en train de révolutionner la pratique de la dermatologie. Il s'agit de la microscopie confocale par réflectance in vivo ou ex vivo, et de la tomographie par cohérence optique, trois techniques complémentaires qui deviennent indispensables pour la prise en charge des cancers cutanés, mais dont l'utilité va bien au-delà.

A Review of Cellular and Acellular Matrix Products: Indications, Techniques, and Outcomes.

BACKGROUND: Wound healing is a dynamic process whereby cells, growth factors (GFs), and the extracellular matrix (ECM) interact to restore the architecture of damaged tissue. Chronic wounds can be difficult to treat due to the increased presence of inflammatory cells that degrade the ECM, GF, and cells necessary for wound healing to occur. Cellular and acellular matrix products can be used in the management of a variety of chronic wounds including venous, diabetic, and pressure ulcers and other conditions such as burns, epidermolysis bullosa, pyoderma gangrenosum, and surgical wounds. These matrices provide cells, GF, and other key elements that act as a scaffold and promote reepithelialization and revascularization of the wound bed. METHODS: This article focuses on cellular and acellular matrix products that have been well-studied clinically with positive results in randomized clinical trials and widely available matrices for chronic nonhealing wounds. We present trial results as well as their indications, techniques, and outcomes. RESULTS: There are a variety of matrix products available on the market. Some of these products are used to treat chronic wounds, for example, diabetic foot ulcers, venous leg ulcers, pyoderma gangrenosum, and pressure ulcers. In this review, we found that wounds of different etiologies have been treated with a variety of matrices, with successful outcomes compared with standard wound care. CONCLUSIONS: Both cellular and acellular matrix products are useful in the management of a variety of chronic wounds. These matrices provide cells, GF, and other key elements that promote reepithelialization and revascularization of the wound bed while preventing degradation of the ECM. The treatment of chronic wounds with matrix products in combination with standard wound care has been proven to aid in wound healing when added to standard of care.

Epidermal skin grafting.

Autologous skin grafts, such as full- and split-thickness, have long been part of the reconstructive ladder as an option to close skin defects. Although they are effective in providing coverage, they require the need for a trained surgeon, use of anaesthesia and operating room and creation of a wound at the donor site. These drawbacks can be overcome with the use of epidermal skin grafts (ESGs), which can be harvested without the use of anaesthesia in an office setting and with minimal to no scarring at the donor site. ESGs consist only of the epidermal layer and have emerged as an appealing alternative to other autologous grafts for the treatment of acute and chronic wounds. In this article, we provide an overview of epidermal grafting and its role in wound management.