Table 1

Clinical coding for EDS and JHS

Read code descriptions (based on pre-1997 nomenclature)EDS type according to the Villefranche criteriaEDS type according to the March 2017 criteriaRead code version 2ICD-10 code
Ehlers-Danlos syndromePGy2.Q79.6
Ehlers-Danlos syndrome type IClassical typeClassical EDSPGy20
Ehlers-Danlos syndrome type IIPGy21
Ehlers-Danlos syndrome type IIIHypermobility typeHypermobile EDS or hypermobility spectrum disorderPGy22
Ehlers-Danlos syndrome type IVVascular typeVascular EDSPGy23
Ehlers-Danlos syndrome type VX-linked typeNo longer classified as EDSPGy24
Ehlers-Danlos syndrome type VIKyphoscoliotic typeKyphoscoliotic EDSPGy25
Ehlers-Danlos syndrome type VIIArthrochalasia type
Dermatosparaxis type
Arthrochalasia EDS
Dermatosparaxis EDS
PGy26
Ehlers-Danlos syndrome type VIIIPeriodontitis typePeriodontal EDSPGy27
Hypermobility syndrome
(JHS according to the Brighton Criteria)
Hypermobility typeHypermobile EDS or hypermobility spectrum disorderN235.728.5
  • EDS, Ehlers-Danlos syndrome; ICD-10, International Statistical Classification of Diseases and Related Health Problems, 10th Revision; JHS, joint hypermobility syndrome.