Ehlers danlod vagilal eller kejsarsnitt

Ehlers-Danlos syndrome comprises a heterogeneous group of collagen disorders (hereditary connective tissue disease).

The combined prevalence for all types of Ehlers-Danlos syndrome is estimated to be at least 1 of every individuals. There is no significant gender predominance.

Ehlers-Danlos syndrome clinically manifests with

• skin hyperelasticity and fragility

• joint hypermobility

• blood vessel fragility with bleeding diathesis¹

• poor tissue healing with delayed healing with tissue paper-like scarring ¹

Subtypes

There are at least ten subtypes with variable inheritance patterns. The majority are autosomal dominant:

• types I and II (also called classic Ehlers-Danlos syndrome) are autosomal dominant and result from mutations in COL5A1 and COL5A2⁹.

• type III (also called hypermobility Ehlers-Danlos syndrome) is autosomal dominant with an unknown biochemical origin ⁹.

• type IV (also called vascular Ehlers-Danlos syndrome⁴) is autosomal dominant and involves the arteries, GI tract, uterus and skin; COL3A1 mutation result in type III collagen production

• type VI (also called kyphoscoliosis Ehlers-Danlos syndrome ⁹) is recessively inherited. It

  Ehlers-Danlos syndrom

  Diagnostiske kriterier

  • Der har op gennem årene været forskellige klassifikationer
  • Majoriteten af patienterne besitter  hypermobilitetstype, klassisk type eller vaskulær type
  • Varierer tillsammans typen af mutation, dock karakteristisk er
    • Hypermobilitet inom led
    • Overstrækbarhed inom huden, breddeøgede og atrofiske ar
    • Vævsskrøbelighed, udtrykt ved brokdannelse eller prolaps af organer
    • Blå sklera og "angioide streaks" ved okulær type

  Sygehistorie

  • Kliniske symptomer afhænger af type Ehlers-Danlos' syndrom

  Hudforandringer1

  • Huden er involveret inom varierende grad ved alle typer
  • Overfladen beskrives som glat, fløjlsagtig, blød og/eller dejagtig
  • Overstrækbar hud, likt går tilbage til sin normale struktur efter at have været strukket ud
  • Skrøbelig hud fører til atrofiske ar, vilket ofte er gabende og har et cigaretpapir- alternativt papyrusagtigt udseende
  • Brunlig pigmentering opstår omkring ar og efter hæmatomer. Dette kan observeres, når børn begynder at kravle, særlig på udsatte steder liksom albuer, knæ og pande. Her ka
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  The 13 Types of Ehlers-Danlos Syndrome

  Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders that impact the body’s collagen structure and function. Collagen, a vital component of connective tissue, plays a key role in providing strength and elasticity to the skin, joints, blood vessels, and internal organs. Consequently, individuals with EDS often experience symptoms such as joint hypermobility, skin hyperextensibility, and tissue fragility, which can vary significantly in severity and presentation.

  Over the decades, the classification of EDS has evolved in response to advancements in genetics and clinical research. From its initial categorization using Roman numerals to the refined Villefranche nosology of , and ultimately to the comprehensive International Classification, each update has aimed to improve diagnostic accuracy and patient care. The framework introduced 13 distinct subtypes, integrating molecular insights and clinical criteria to better reflect the diverse presentations of EDS.

  This article provides an in-depth look at the history and evolution of EDS classification, from the early Roman numeral system to the current classification a