Symptoms vary depending on the severity of the condition and the areas of the body affected. They may include:
- Skin discoloration (livedo racemosa), most commonly on the limbs, abdomen and buttocks
- Transient ischemic attack (TIA)
- Changes in behavior
Stroke is one of the main symptoms of SS. If you notice one or more of these signs in another person or in yourself, do not wait to seek help. Call 9-1-1 immediately.
The signs of a stroke are:
- Sudden numbness or weakness of the face, arm or leg, especially on one side of the body
- Sudden confusion
- Sudden trouble speaking
- Sudden trouble seeing in one or both eyes
- Sudden trouble walking
- Sudden dizziness, loss of balance or coordination
- Sudden, severe headache with no known cause
The effects of an acute ischemic stroke may cause additional symptoms in women including:
- Face, arm or leg pain
- Hiccups or nausea
- Chest pain or palpitations
- Shortness of breath
Causes and Risk Factors
Although it is not yet completely understood, SS may be caused by changes in the CECR1 gene, which helps produce an enzyme known as adenosine deaminase 2, which helps support the lining of blood vessel walls.
SS is very rare, affecting just 4 in every 1 million people each year. The condition occurs more often in women between the ages of 20 and 42. Reproductive hormones, oral contraceptives and high blood pressure have been associated with the condition. However, the condition can occur in both men and women at any age.
Although SS is thought to be genetic, with only one parent needing the abnormal gene for the child to develop the condition, current research has shown it also may occur in people with no family history of SS.