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Ehlers-Danlos syndrome

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Disease definition

A heterogeneous group of diseases characterized by fragility of the soft connective tissues resulting in widespread skin, ligament, joint, blood vessel and/or internal organ manifestations. Clinical spectrum is highly variable, ranging from mild skin and joint hyperlaxity to severe physical disability and life-threatening vascular complications. Overlap with osteogenesis imperfecta may be observed resulting in an EDS/osteogenesis imperfecta overlap phenotype. Diseases in this group include classical Ehlers-Danlos syndrome (EDS), musculocontractural EDS, hypermobile EDS, vascular EDS, arthrochalasia EDS, dermatosparaxis EDS, periodontal EDS, X-linked EDS, brittle cornea syndrome, classical-like EDS type 1 and type 2, cardiac-valvular EDS, spondylodysplastic EDS, myopathic EDS, and kyphoscoliotic EDS.

ORPHA:98249

Classification level: Group of disorders

Synonym(s):
  • EDS

Prevalence: Unknown

Inheritance: Autosomal dominant, Autosomal recessive, X-linked recessive

Age of onset: Infancy, Neonatal

ICD-10: Q79.6

ICD-11: LD28.1

UMLS: C0013720

MeSH: D004535

GARD: 6322

MedDRA: 10014316

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2017.pdf) - Orphanet Urgences
Polski (2009.pdf) - Orphanet Urgences
Deutsch (2009.pdf) - Orphanet Urgences
English (2009.pdf) - Orphanet Urgences
Español (2009.pdf) - Orphanet Urgences
Italiano (2009.pdf) - Orphanet Urgences
Português (2009.pdf) - Orphanet Urgences
Anesthesia guidelines
Deutsch (2019) - Orphananesthesia
English (2019) - Orphananesthesia
Español (2019) - Orphananesthesia
Čeština (2019) - Orphananesthesia
Clinical practice guidelines
English (2018) - RMD Open
English (2014) - Orphanet J Rare Dis Logo ERN
English (2020) - Nat Rev Dis Primers Logo ERN
English (2018) - RMD Open
Disease review articles
Review article
Français (2023) - Bull Cancer
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.