Chilblain Lupus

What is chilblain lupus?

Chilblain lupus is a symptom of lupus. Lupus is an autoimmune disorder (when your immune system mistakenly attacks healthy cells). People with chilblain lupus get painful red or purple sores and discolored patches of skin. These small sores (chilblains) form or worsen after exposure to cold temperatures. The word “chilblain” comes from words that mean “cold” and “sore.”

Are all chilblains lupus?

No. Lupus can cause chilblains, but not all chilblains are lupus. You have a higher risk of chilblains if you:

• Have poor blood circulation.
• Wear tight-fitting shoes and clothes
• Live in a humid, cold climate.

Who might get chilblain lupus?

Anyone can develop chilblain lupus, but it is most common in adults. People who live in cold, wet climates may be more likely to have chilblain lupus.

Some conditions can increase a person’s chances of getting chilblain lupus, including:

• Anorexia nervosa, a life-threatening eating disorder.
• Intestinal lymphoma, a rare type of lymphoma (lymph system cancer) that starts in your small bowel.
• Pregnancy.

How common is chilblain lupus?

Chilblain lupus is rare, although healthcare providers know it is underreported. Because many people don’t seek medical attention, providers don’t know how widespread it is. In 2008, one literature review found only 70 reported diagnoses of chilblain lupus.

Can I have more than one type of lupus?

When people refer to lupus, they usually mean systemic lupus erythematosus (SLE). About 7 out of every 10 people who have lupus have SLE.

What causes chilblain lupus?

Chilblain lupus can be inherited or sporadic. Sporadic chilblain lupus means you develop the condition later in life, usually as an adult. Doctors don’t know why it occurs.

People with inherited chilblain lupus have a gene mutation (change). A change in either of these genes can lead to chilblain lupus:

• SAMHD1 genes play a role in your immune response.
• TREX1 helps with DNA repair.

What are the symptoms of chilblain lupus?

Chilblain lupus causes red or purple skin patches or sores. Usually, these sores appear on your:

• Fingers.
• Heels.
• Soles of your feet.
• Toes.

Less commonly, you may get sores on the palms of your hands, knees, nose or ears. Typically, these sores appear or worsen in cold or wet weather.

Chilblain symptoms may also include:

• Blisters or ulcers, open sores caused by poor blood circulation.
• Depigmentation, patches of skin lightening or losing color.
• Heel fissures, deep cracks in the skin on your heels.
• Hyperkeratosis, skin thickening.
• Raynaud’s phenomenon, temporary blood restriction in your fingers and toes.

What are the complications of chilblain lupus?

Without treatment, chilblain lupus skin lesions can get infected. People with chilblain lupus have higher risks of bacterial skin infections (cellulitis).

How is chilblain lupus diagnosed?

A dermatologist (skin care doctor) can diagnose chilblain lupus. Usually, dermatologists use a test called a skin biopsy.

During a skin biopsy, your dermatologist takes a small skin sample. Your doctor sends this sample to a laboratory, where specialists look at it under a microscope. Examining skin under a microscope can tell healthcare providers whether chilblain lupus or another condition is causing skin sores.

How is chilblain lupus treated?

For mild chilblain lupus symptoms, you may only need to protect yourself from cold temperatures. If you smoke, your provider will also encourage you to quit. Smoking constricts your blood vessels, which can make chilblain lupus symptoms worse.

Your provider may prescribe medications for moderate to severe chilblain lupus. Common medications include:

• Antibiotics, such as dicloxacillin (Dycill®) or erythromycin (Ilosone®), treat bacterial skin infections.
• Antimalarial agents, such as hydroxychloroquine (Plaquenil®), fight fatigue, reduce inflammation and decrease joint pain.
• Calcineurin inhibitors, such as tacrolimus (Prograf™) or mycophenolate mofetil (CellCept®), decrease inflammation by lowering your immune system response.
• Calcium channel blockers, such as nifedipine (Adalat®), encourage your blood vessels to open.
• Steroid ointments, such as fluocinolone acetonide (Synalar®) or hydrocortisone butyrate (Locoid®), decrease swelling and lower inflammation.
• Systemic steroids, such as prednisone (Deltasone®), are swallowable pills that reduce inflammation and swelling.

Do I need surgery for chilblain lupus?

Rarely, your provider may recommend surgery to treat chilblain lupus. Your provider may surgically remove chilblain sores and perform a skin graft. During a skin graft, your provider takes a small amount of healthy skin from one part of the body and safely transfers it to another.

How can I prevent chilblain lupus symptoms?

You can reduce chilblain lupus flare-ups by protecting yourself from cold temperatures. You may:

• Exercise regularly to improve blood flow and regulate your body temperature.
• Make sure your home and workspace are insulated and heated.
• Soak your hands or feet in warm water several times a day.
• Warm cold hands or feet gradually to encourage blood flow to return.
• Wear thick wool socks, gloves and warm shoes whenever you’re in a cold environment.

What is the outlook for people with chilblain lupus?

Chilblain lupus is a lifelong condition. With treatment and proper cold protection, many people manage symptoms well.

What else should I ask my healthcare provider?

You may also want to ask your healthcare provider:

• What is the most likely cause of chilblain lupus?
• Am I likely to develop systemic lupus erythematosus?
• How can I prevent chilblain lesions from developing?
• How can I treat existing chilblain lesions?

A note from Cleveland Clinic

Chilblain lupus is a rare complication of discoid lupus (lupus that affects your skin). The condition causes painful red or purplish sores, usually on your fingers or feet. Typically, these sores develop or worsen after exposure to cold weather. Your provider may prescribe medications to lower inflammation and swelling. You may prevent chilblain sores by keeping warm in cold environments. With treatment and cold protection, many people with chilblain lupus manage symptoms well.