A5009387893- Skin and Cellular Biology Research
- Autoimmune Bullous Skin Diseases
- Cell Adhesion Molecules Research
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- Wnt/β-catenin signaling in development and cancer
- Cellular Mechanics and Interactions
- Cancer Cells and Metastasis
- Dermatology and Skin Diseases
- Eosinophilic Disorders and Syndromes
- Silk-based biomaterials and applications
- Mesenchymal stem cell research
- Polysaccharides and Plant Cell Walls
- Viral-associated cancers and disorders
- Acute Lymphoblastic Leukemia research
- Nail Diseases and Treatments
- Microtubule and mitosis dynamics
- Transgenic Plants and Applications
- Biochemical and Molecular Research
- Cardiovascular Function and Risk Factors
- Contact Dermatitis and Allergies
- Cancer Research and Treatments
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Immunodeficiency and Autoimmune Disorders
- Dermatological and Skeletal Disorders
- Liver Disease Diagnosis and Treatment
Inserm
2007-2024
Institut des Maladies Génétiques Imagine
2018-2024
Université Paris Cité
2020-2024
Sorbonne Paris Cité
2020
Institut de Génétique Moléculaire de Montpellier
2007
Netherton syndrome (NS) is a rare, severe genetic disorder of cornification with high morbidity. Treatment for NS has been notoriously difficult. Recent studies showed an upregulated helper T cell (TH) 17/interleukin 23 (IL-23) pathway in NS, suggesting the possibility treatment strategies that target IL-17.To evaluate clinical response to IL-17 antagonist secukinumab.This case series study reports experience compassionate use therapy secukinumab 4 patients including 2 children, from...
BackgroundNetherton syndrome (NS) is a rare recessive skin disorder caused by loss-of-function mutations in SPINK5 encoding the protease inhibitor LEKTI (lymphoepithelial Kazal-type–related inhibitor). NS patients experience severe barrier defects, display inflammatory lesions, and have superficial scaling with atopic manifestations. They present typical ichthyosis linearis circumflexa (NS-ILC) or scaly erythroderma (NS-SE).ObjectiveWe used combination of several molecular profiling methods...
Recessive dystrophic epidermolysis bullosa (RDEB) is a rare severe hereditary skin disease characterized by and mucosa fragility, resulting in blister formation. The most complication RDEB patients the development of cutaneous squamous cell carcinoma (SCC) leading to premature death. Multiple evidences suggest permissive tumor micro-environment (TME) as driver SCC patients. In cohort patients, we immune profiles RDEB-SCCs compared them with clinical, histopathological, prognostic features....
Recessive dystrophic epidermolysis bullosa (RDEB) is a rare severe hereditary skin disease characterized by and mucosa fragility, resulting in blister formation. The most complication RDEB patients the development of cutaneous squamous cell carcinoma (SCC), leading to premature death. There great deal evidence suggesting permissive tumor microenvironment (TME) as driver SCC patients. In cohort patients, we immune profiles RDEB-SCCs compared them with clinical, histopathological, prognostic...