Ver el código de «Ehlers-Danlos syndrome»

==Background==
*Hereditary [[connective tissue disorder]]
*Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis
*Causes increased risk of:
**[[Thoracic Aortic Aneurysm|Thoracic]] and [[abdominal aortic aneurysm]]
**[[Mitral valve prolapse]]
**Spontaneous [[pneumothorax]]
**Myopia and [[retinal detachment]]
**Premature degenerative [[arthritis]]

==Clinical Features==
[[File:Hyperelastic skin in a case of Ehlers-Danlos syndrome (cropped).png|thumb|Individual with EDS displaying skin hyperelasticity.]]
[[File:Ehlers-Danlos thumb.jpg|thumb|Individual with EDS displaying hypermobile thumb.]]
*Type I and II: Classical
**Hyperextension of the joints and skin
**Easy bruising and skin fragility
**Congenital dislocations of the hips, recurrent dislocations of other joints
**Spondolithesis
**Joint effusions
**Club foot
*Type III: Hypermobility
**Similar to Classic, however less skin involvement
*Type IV: Vascular
**Colonic perforation
**Ruptured arteries
**Uterine rupture during pregnancy
**Severe and frequent wound dehiscence after surgery
**Only type that significantly affects mortality
*Type VI: Kyphoscoliotic
**Fragility of the ocular globe
**Marked joint hyperextension
**Severe scoliosis
*Type VIIA and VIIB: Arthrochalasia
**Profound joint hyperextension
**Moderate short stature
*Type VIIC: Dermatosparaxis
**Minimal joint involvement
**Primarily affects the skin and fascia

==Differential Diagnosis==
*[[Marfan syndrome]]
*Cutis laxa
*Loeys-Dietz syndrome
*Osteogenesis imperfecta
*Larsen Syndrome
*Stickler syndrome
*Arteria tortuosity syndrome

==Evaluation==


==Management==
*Cardiac evaluation to screen for valvular disease, vascular disease, and aortic aneurysm
*Ophthalmologic evaluation to screen for myopia, retinal detachment, and glaucoma
*Monitoring of skin fragility, wound healing, easy bruising

==Disposition==


==See Also==
*[[Connective tissue disease]]

==External Links==


==References==
<references/>

[[Category:Dermatology]]