Hypermobile Ehlers Danlos Syndrome: Difference between revisions
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(Created page with "==Diagnostic Criteria== ===Criterion 1: Generalised Joint Hypermobility=== Must meet Beighton Score for age {| class="wikitable" |- |Age |Beighton Score |- |Prepubescent or ad...") |
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|Patients with Acquired joint limitations | |Patients with Acquired joint limitations | ||
|BS 1 point under age requirements AND a positive 5PQ | |BS 1 point under age requirements AND a positive 5PQ | ||
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===Criterion 2: At least 2 features must be present=== | ===Criterion 2: At least 2 features must be present=== | ||
'''Feature A:'''Systemic manifestations of CTD (need ≥5)' | '''Feature A:'''Systemic manifestations of CTD (need ≥5)' | ||
# ''Unusually soft/velvety skin'' | # ''Unusually soft/velvety skin'' | ||
# ''Mild skin hyperextensibility'' | # ''Mild skin hyperextensibility'' | ||
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'''Feature C: '''MSK Complications(need ≥1) | '''Feature C: '''MSK Complications(need ≥1) | ||
# MSK pain in ≥2 limbs, recurring daily for ≥3 months | # MSK pain in ≥2 limbs, recurring daily for ≥3 months | ||
# Chronic widespread pain for ≥3 months | # Chronic widespread pain for ≥3 months | ||
# Recurrent joint dislocations or frank joint instability, in the absence of trauma (a or b) | # Recurrent joint dislocations or frank joint instability, in the absence of trauma (a or b) | ||
# | #* ≥3 atraumatic dislocations in same joint or ≥2 more atraumatic dislocations in two difference joints occurring at different times | ||
# | #* Medical confirmation of joint instability at two or more sites not related to trauma | ||
===Criterion 3: All three prerequisites must be met=== | ===Criterion 3: All three prerequisites must be met=== | ||
#Absence of unusual skin fragility. | |||
#Exclusion of other heritable and acquired connective tissue disorders. In patients with an acquired onnective tissue disorder, additional diagnosis of hEDS requires meeting both Features A and B of Criterion 2. Feature C of Criterion 2 cannot be counted in this situation. | |||
#Exclusion of alternative diagnoses that may also include joint hypermobility by means of hypotonia and/or connective tissue laxity | |||
Revision as of 21:53, 19 June 2020
Diagnostic Criteria
Criterion 1: Generalised Joint Hypermobility
Must meet Beighton Score for age
| Age | Beighton Score |
| Prepubescent or adolescent | ≥6 |
| Pubescent up until age 50 | ≥5 |
| Over age 50 | ≥4 |
| Patients with Acquired joint limitations | BS 1 point under age requirements AND a positive 5PQ |
Criterion 2: At least 2 features must be present
Feature A:Systemic manifestations of CTD (need ≥5)'
- Unusually soft/velvety skin
- Mild skin hyperextensibility
- Unexplained striaedistensae/rubrae
- Bilateral piezogenic papules of heel
- Recurrent/multiple abdominal hernia
- Atrophic scaring in ≥2 sites
- Pelvic floor, rectal, and/or uterine prolapse in children, men or nulliparous women
- Dental crowding and high or narrow palate
- Arachnodactyly
- Arm span-to-height ≥1.05
- Mitral valve prolapse
- Aortic root dilatation with Z score > +2
Feature B: Family history (1 or more first-degree relatives must meet criteria)
Feature C: MSK Complications(need ≥1)
- MSK pain in ≥2 limbs, recurring daily for ≥3 months
- Chronic widespread pain for ≥3 months
- Recurrent joint dislocations or frank joint instability, in the absence of trauma (a or b)
- ≥3 atraumatic dislocations in same joint or ≥2 more atraumatic dislocations in two difference joints occurring at different times
- Medical confirmation of joint instability at two or more sites not related to trauma
Criterion 3: All three prerequisites must be met
- Absence of unusual skin fragility.
- Exclusion of other heritable and acquired connective tissue disorders. In patients with an acquired onnective tissue disorder, additional diagnosis of hEDS requires meeting both Features A and B of Criterion 2. Feature C of Criterion 2 cannot be counted in this situation.
- Exclusion of alternative diagnoses that may also include joint hypermobility by means of hypotonia and/or connective tissue laxity
