Classic EDS is mainly characterized by skin hyperextensibility, abnormal wound healing, and ligament hypermobility.
This type of EDS is characterized by the involvement of the following organs and tissues.
Skin in people affected with this type of EDS may have some of the following characteristics:
- Easily stretched, returns to its initial position when released (skin hyperextensibility).
- Soft and velvety texture.
- Fragile, easily bruised and damaged.
- Delayed wound healing.
- Bluish hands and feet (acrocyanosis).
- Lesions can appear at pressure sites such as the elbow and knee (moluscoid pseudotumors).
- Small nodules or cysts, often movable, can be detected under the skin of forearms and shins (subcutaneous spheroids).
- Protrusions in the heels may be visible when standing up and disappear when the feet are lifted (piezogenic papules).
- Chilblains, injuries characterized by painful or itchy skin inflammation, are also frequent.
Multiple organs can be affected:
- Cervical insufficiency during pregnancy, which may lead to inability to carry a pregnancy to term or until the fetus is viable.
- Inguinal, umbilical, incisional, and hiatal hernias.
- Recurrent rectal prolapse during early infancy.
Complications due to joint hypermobility:
- Dislocation of the shoulder, patella, fingers, hips, radius, and collarbones, usually spontaneously resolved or easily reduced by the affected individual.
- Feet deformities, such as congenital clubfoot or flatfoot, temporomandibular joint dysfunction, and osteoarthritis.
Primary muscle hypotonia may occur, leading to delayed motor development, ambulation problems, and minor motor alterations.
Fatigue and muscle cramps are relatively frequent and, in some cases, cerebrospinal fluid leaks have been identified as the cause of orthostatic hypotension and headache in individuals with classic EDS.
Structural cardiac malformations are rare in classic EDS.