Rare dermatological diseases cause great difficulties for sufferers. They impact their lives through visibility, pain, restrictive care and sometimes serious complications. This article describes the management of three rare dermatological diseases by caregivers trained and sensitized to these pathologies in centers of reference for rare dermatological diseases.
View Article and Find Full Text PDFBackground: Epidermal necrolysis (EN) involving ≥10% of the body surface area (BSA) is often complicated by bacterial infections.
Objective: We sought to describe the epidemiology of bloodstream infections (BSIs) in EN involving a BSA ≥10% and the diagnostic performances of skin cultures for predicting the pathogen(s) isolated from BSIs.
Methods: This retrospective single-center observational study was conducted between 2009 and 2017.
Epidermal necrolysis (EN) encompasses Stevens-Johnson syndrome (SJS, < 10% of the skin affected), Lyell syndrome (toxic epidermal necrolysis, TEN, with ≥30% of the skin affected) and an overlap syndrome (10 to 29% of the skin affected). These rare diseases are caused, in 85% of cases, by pharmacological treatments, with symptoms occurring 4 to 28 days after treatment initiation. Mortality is 20 to 25% during the acute phase, and almost all patients display disabling sequelae (mostly ocular impairment and psychological distress).
View Article and Find Full Text PDFCyclosporine has shown promising results for mortality in patients with Stevens-Johnson syndrome/toxic epidermal necrolysis. However, available studies included only a small number of patients and did not include a validated and homogenous control group. We present the results from a retrospective monocentric study including 174 patients with Stevens-Johnson syndrome/toxic epidermal necrolysis during 2005-2016.
View Article and Find Full Text PDFToxic epidermal necrolysis (TEN) is a severe cutaneous adverse drug reaction leading to extensive sloughing of the skin. Late cutaneous complications such as pigmentation disorders are frequently reported. In this report, we present particular facial cutaneous sequelae with histological analysis after TEN.
View Article and Find Full Text PDFOwing to its phylogenetic position at the base of the chordates, the cephalochordate amphioxus is an emerging model system carrying immense significance for understanding the evolution of vertebrate development. One important shortcoming of amphioxus as a model organism has been the unavailability of animal husbandry protocols to maintain amphioxus adults away from the field. Here, we present the first report of successful maintenance and spawning of Branchiostoma lanceolatum adults in a facility run on artificial seawater.
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