ICD-10-CM Code for Ehlers-Danlos Syndrome
The ICD-10-CM code for Ehlers-Danlos syndrome is Q79.6. 1
Code Details and Clinical Context
Q79.6 is the single diagnostic code used for all subtypes of Ehlers-Danlos syndrome in the ICD-10-CM classification system. 1
This code replaced the ICD-9 code 756.83, which was previously used for EDS diagnosis prior to the transition to ICD-10-CM. 1
Important Clinical Considerations When Using This Code
Subtype specification is critical despite using a single ICD code, as the 13 different EDS subtypes have dramatically different clinical implications, prognoses, and management strategies. 2, 3
Vascular EDS (Type IV) carries life-threatening risks with median survival of 48 years due to arterial rupture, requiring urgent COL3A1 genetic testing for confirmation, yet uses the same Q79.6 code as benign hypermobile variants. 2, 4
Hypermobile EDS represents 80-90% of all EDS cases and has no genetic test available, requiring clinical diagnosis based on the 2017 criteria including Beighton score ≥5/9, soft velvety skin, and absence of alternative diagnoses. 4, 5
Documentation Requirements Beyond the ICD Code
Specify the EDS subtype in clinical documentation (vascular, hypermobile, classical, kyphoscoliotic, arthrochalasia, or dermatosparaxis) as this determines screening protocols, surgical risk stratification, and family counseling. 3, 6
Document genetic confirmation status, particularly whether COL3A1 pathogenic variants have been identified for vascular EDS or COL5A1/COL5A2 for classical EDS, as molecular confirmation changes management. 4, 1
Note associated complications separately with additional ICD codes, such as aortic root dilation (25-33% of hypermobile/classical EDS), POTS (up to 37.5% of hypermobile EDS), or gastrointestinal manifestations (98% of hypermobile EDS). 4, 5
Common Coding Pitfalls to Avoid
Do not use Q79.6 based on clinical suspicion alone without applying validated diagnostic criteria (Beighton score, skin assessment, family history) or genetic testing when indicated, as 26.4% of clinically suspected cases have alternative genetic conditions requiring different management. 5
Avoid coding EDS in patients with isolated joint hypermobility (Beighton score ≥5/9) without the additional required features of skin involvement and exclusion of alternative diagnoses, as joint hypermobility alone does not constitute EDS. 4, 5
Never code vascular EDS without molecular confirmation of COL3A1 pathogenic variants, as clinical criteria alone have poor specificity with high overlap with other arteriopathies, leading to inappropriate surveillance and treatment strategies. 1