Ehlers Danlos syndrome (EDS): classification and differentials

EDS types

• Classical (hypermobile) EDS
• Classical-like
• Cardiac-valvular (cvEDS)
• Vascular
• Hypermobile
• Arthrochalasia
• Dermatopraxis (dEDS)
• Kyphoscoliotic (kEDS)
• Brittle cornea syndrome (BCS)
• Spondylodysplastic (spEDS)
• Musculocontractural (mcEDS)
• Myopathic (mEDS)
• Periodontal (pEDS)

Differential diagnosis

References

1. Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017; 175:8-26. 
2. Brady AF, Demirdas S, Fournel-Gigleux S, et al. The Ehlers-Danlos syndromes, rare types. Am J Med Genet C Semin Med Genet 2017; 175:70-115.
3. Castori M, Voermans NC. Neurological manifestations of Ehlers-Danlos syndrome(s): a review. Iran J Neurol 2014; 13:190-208. 
4. Donkervoort S, Bonnemann CG, Loeys B, Jungbluth H, Voermans NC. The neuromuscular differential diagnosis of joint hypermobility. Am J Med Genet C Semin Med Genet 2015; 169C:23-42. 
5. Colombi M, Dordoni C, Chiarelli N, Ritelli M. Differential diagnosis and diagnostic flow chart of joint hypermobility syndrome/ehlers-danlos syndrome hypermobility type compared to other heritable connective tissue disorders. Am J Med Genet C Semin Med Genet 2015; 169C:6-22.
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Related checklists

• Ehlers Danlos syndrome (EDS): neurological features
• Ehlers Danlos syndrome (EDS): systemic features
• Ehlers Danlos syndrome (EDS): management