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Start free with EleplanArthrochalasia Ehlers-Danlos syndrome
ORPHA:1899Disease
Also called Arthrochalasia EDS · Arthrochalasis multiplex congenita · EDS VII · Ehlers-Danlos syndrome type 7 · Ehlers-Danlos syndrome, arthrochalasia type · aEDS
What it is
A form of Ehlers-Danlos syndrome (EDS) characterized by congenital bilateral hip dislocation, severe generalized joint hypermobility with recurrent joint dislocations and subluxations, hyperextensible and/or fragile skin.
Key facts
- Age of onset
- Infancy, Neonatal
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 1 000 000 (at birth, Europe)Ehlers-Danlos syndrome
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Very common80–99%
10- Avascular necrosis of the capital femoral epiphysis
- Congenital bilateral hip dislocationDiagnostic criterion
- Delayed ability to walk
- Generalized joint hypermobilityDiagnostic criterion
- Hip dislocation
- Hip dysplasia
- Hyperextensible skinDiagnostic criterion
- Motor delay
- Multiple joint dislocationDiagnostic criterion
- Thin skin
Common30–79%
17Sometimes5–29%
19- Atrophic scarsDiagnostic criterion
- Blue sclerae
- Breech presentation
- Bruising susceptibilityDiagnostic criterion
- Cleft lip
- Cleft palate
- Decreased fetal movement
- Dentinogenesis imperfecta
and 11 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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