Publications by authors named "B Sans"
Background: Telangiectasia macularis eruptiva perstans (TMEP) has not been fully characterized.
Objective: We sought to estimate the frequency and clinical characteristics of TMEP in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement.
Methods: We included all consecutive patients evaluated for cutaneous mastocytosis in 2 centers: the Mastocytosis Competence Center of the Midi-Pyrénées from May 2006 to December 2013, and the French Reference Center for Mastocytosis from January 2008 to September 2013.
Treatment options for patients suffering from indolent forms of mastocytosis remain inadequate with the hyperactivation of mast cells responsible for many of the disease's systemic manifestations. Masitinib is a potent and highly selective oral tyrosine kinase inhibitor. A combined inhibition of c-Kit and Lyn make it particularly efficient in controlling the activity of mast cells and therefore, of potential therapeutic benefit in mastocytosis.
View Article and Find Full Text PDFAdult mastocytosis is an incurable clonal disease associated with c-KIT mutations, mostly in exon 17 (D816V). In contrast, pediatric mastocytosis often spontaneously regresses and is considered a reactive disease. Previous studies on childhood mastocytosis assessed only a few patients and focused primarily on codon 816 mutations, with various results.
View Article and Find Full Text PDFBackground: Indolent forms of mastocytosis account for more than 90% of all cases, but the types and type and severity of symptoms and their impact on the quality of life have not been well studied. We therefore performed a case-control cohort study to examine self-reported disability and impact of symptoms on the quality of life in patients with mastocytosis.
Methodology/principal Findings: In 2004, 363 mastocytosis patients and 90 controls in France were asked to rate to their overall disability (OPA score) and the severity of 38 individual symptoms.
Article Synopsis
- Adult mastocytosis tends to have ongoing systemic involvement with a higher prevalence of c-kit 816 mutations, while pediatric mastocytosis is usually skin-limited and can resolve on its own.
- A study of 142 adult patients revealed that systemic disease percentages were similar in those with childhood-onset compared to adult-onset mastocytosis, but a significant difference in c-kit mutation rates was observed.
- The findings suggest that the development of mastocytosis varies based on age at onset, which could be crucial for future c-kit-targeted treatments.