Preferred Label : Ehlers-Danlos syndrome, vascular type;
ICD-11 definition : Ehlers-Danlos syndrome type IV, also known as the vascular type of Ehlers-Danlos syndrome
(EDS), is an inherited connective tissue disorder defined by characteristic facial
features (acrogeria) in most patients, translucent skin with highly visible subcutaneous
vessels on the trunk and lower back, easy bruising, and severe arterial, digestive
and uterine complications, which are rarely, if at all, observed in the other forms
of EDS.;
ICD-11 synonym : Sack-Barabas syndrome; Ehlers-Danlos syndrome type 4;
Origin ID : 1202686415;
UMLS CUI : C0268338;
Currated CISMeF NLP mapping
Semantic type(s)
UMLS correspondences (same concept)
Validated automatic mappings to NTBT
Ehlers-Danlos syndrome type IV, also known as the vascular type of Ehlers-Danlos syndrome
(EDS), is an inherited connective tissue disorder defined by characteristic facial
features (acrogeria) in most patients, translucent skin with highly visible subcutaneous
vessels on the trunk and lower back, easy bruising, and severe arterial, digestive
and uterine complications, which are rarely, if at all, observed in the other forms
of EDS.