Severe Mast Cell Activation Syndrome in a 15-year-old patient with an hypermobile Ehlers-Danlos syndrome
Abstract
We report the history of a 15-year old patient with a hypermobile Ehlers-Danlos syndrome (hEDS) (his mother, his two brothers and his sister have the same phenotype as him). He suffers mainly from a severe mast cell activation syndrome (MCAS) with an overreaction of the skin to any kind of contact (water of the shower, clothes, bed sheets) but he has also fatigue, headaches, and rash. This impressive rash is exacerbated after the shower and he has the urge to rest («shower's sign»). We describe the MCAS and its easy, fast and very effective medication management, without any significant side effects as well as its frequent association with the hEDS. We finally introduce the original term of «MASED» to this MCAS, associated, linked or entangled to hEDS.
Nous présentons le cas d’un jeune patient âgé de 15 ans atteint d’un syndrome d’Ehlers-Danlos (SED) de type hypermobile (sa mère, ses deux frères et sa soeur présentent le même phénotype que lui). Il présente principalement un syndrome d’activation mastocytaire (SAMA) sévère avec une atteinte démesurée au niveau de la peau exposée au simple contact (avec l’eau, les draps, les vêtements), mais également de la fatigue, des céphalées ainsi que des éruptions qui sont exacerbées après la douche avec l’envie impérieuse de se reposer (le «signe de la douche»). Nous décrivons le SAMA, sa prise en charge médicamenteuse simple, rapide et efficace et dépourvue d’effets secondaires notables ainsi que son association fréquente au SED. Nous…
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Artificial Intelligence in Medicine: Cross-Sectional Study Among Medical Students on Application, Education, and Ethical Aspects
- Information quality of videos related to esophageal cancer on tiktok, kwai, and bilibili: a cross-sectional study
- The quality and reliability of short videos about hypertension on TikTok: a cross-sectional study
- Quality and reliability evaluation of pancreatic cancer-related video content on social short video platforms: a cross-sectional study
- Quality and reliability of pediatric pneumonia related short videos on mainstream platforms: cross-sectional study
- Association between metabolic score for insulin resistance (METS-IR) and hypertension: a cross-sectional study based on NHANES 2007–2018
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.