Sneddon Syndrome

What is Sneddon syndrome?

Sneddon syndrome is a disease that affects your small and medium blood vessels. The endothelium (inner lining) of your vessels grows too quickly and the excess number of cells creates blood flow problems.

Sneddon syndrome is rare and progressive (it worsens over time). If you have this disease, you’re likely to have blood clots, and skin and neurological issues.

Other names for Sneddon syndrome are Sneddon-Champion syndrome and livedo reticularis racemosa. It may also cause strokes and transient ischemic attacks (TIAs).

How common is Sneddon syndrome?

Sneddon syndrome affects an estimated 4 per 1 million people per year (or 1 in 250,000 people).

Sneddon syndrome happens more often in women than in men. Nearly 80% of people with Sneddon syndrome are women. They’re usually diagnosed around the age of 40.

What are the signs and symptoms of Sneddon syndrome?

People with Sneddon syndrome may have signs and symptoms that include:

• Painless rash that looks like fish nets or the patterns found on some snakes. The rash is called livedo reticularis if it’s on your legs or arms and appears when you’re cold and goes away when you’re warmer. If the rash is on your trunk and buttocks and persists even if you’re warm, it’s called livedo racemosa.
• Headaches.
• Dizziness.
• Strokes and TIAs.
• Changes in your personality, and your ways of thinking and behaving.
• Kidney problems (rare).

The signs and symptoms don’t usually appear all at once. You may develop purplish or reddish skin colorings years before any type of neurological events happen. Or you might have issues with your eyes or headaches first.

What causes Sneddon syndrome?

Researchers don’t know what causes Sneddon syndrome (so, it’s called idiopathic). It has appeared repeatedly in some families, so it may have a genetic component.

In some cases, Sneddon syndrome happens along with some autoimmune conditions. Autoimmune diseases happen when your body attacks itself. If you have Sneddon syndrome, you might also have:

• Lupus.
• Antiphospholipid syndrome.
• Behçet’s disease.
• Mixed connective tissue disease.

How is Sneddon syndrome diagnosed?

If your healthcare provider suspects you have Sneddon syndrome, they’ll order various tests on:

• Your blood, to look for signs of autoimmune disease or clotting issues.
• Your heart, to examine blood flow in your heart and blood vessels.
• Your brain, to look for damage.
• Your skin, to look for changes on a skin biopsy that are characteristic of the disease.

These tests may include imaging tests like magnetic resonance imaging (MRI) scans, computed tomography (CT) scans and angiograms, as well as skin biopsy.

How is Sneddon syndrome treated?

The best way to treat Sneddon syndrome isn’t yet defined, partly because there aren’t enough people with the condition to have large clinical trials.

The different features of the disease warrant different treatments. For instance, your healthcare provider may prescribe:

• Anticoagulant or antiplatelet medications to help blood flow.
• Vasodilators like nifedipine to increase blood flow. This generally helps skin symptoms.
• Angiotensin-converting enzyme inhibitors (ACE inhibitors) to stop the growth of endothelial cells.

You shouldn’t smoke or use estrogen-containing contraceptives if you have Sneddon syndrome.

How can I prevent Sneddon syndrome?

There’s no way to prevent Sneddon syndrome.

What can I expect if I have Sneddon syndrome?

In many cases, Sneddon syndrome causes neurological changes like memory loss and difficulty speaking that progressively worsen. In some cases, people with Sneddon syndrome are diagnosed with early-onset dementia.

Is there a cure for Sneddon syndrome?

Currently, there isn’t a cure for Sneddon syndrome.

When should I contact my healthcare provider about Sneddon syndrome?

You should contact your healthcare provider about any of the symptoms of Sneddon syndrome, like headache or dizziness, especially if you have a skin rash that has a net-like pattern. If you’ve already been diagnosed with Sneddon syndrome, contact your healthcare provider if you notice new symptoms or if you feel like you’re getting worse.

Always call 911 or get to an emergency room if you have symptoms of a stroke:

• Severe headache.
• Trouble with vision.
• Numbness or paralysis on one or both sides of your body.
• Difficulty walking.
• Difficulty talking or understanding what’s being said.

A note from Cleveland Clinic

Sneddon syndrome, though potentially serious, is also very rare. If you’re diagnosed, follow your healthcare provider’s recommendations and make sure you keep your follow-up appointments and testing schedules. You can help yourself by sticking to heart-healthy lifestyle habits and by avoiding smoking and oral contraceptives or other types of estrogen.