Chilblain Lupus

Chilblain lupus is an uncommon type of lupus that affects your skin (cutaneous lupus erythematosus or CLE). Chilblain lupus causes painful red or purple sores and discolored patches (plaques) 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.” For most people, avoiding cold temperatures and taking medications keep symptoms at bay.

For many people, chilblain lupus develops after being diagnosed with the most common type of lupus, systemic lupus erythematosus. Other people get the disease randomly. Either way, the condition can be uncomfortable and debilitating. If you notice itchy, raised patches or plaques, especially on your fingers or toes, reach out to your healthcare provider for treatment.

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 lupus 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

Chilblain lupus causes

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

People with inherited chilblain lupus have a gene variant (change). A change in either the TREX1 or SAMHD1 gene can lead to chilblain lupus. The TREX1 gene helps with DNA repair. The SAMHD1 gene plays a role in your immune response. Both gene mutations can be found in people who have a biological family history of the disease or who develop it as children.

Chilblain lupus risk factors

Anyone can develop chilblain lupus, but it’s 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

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).

In addition, if you haven’t developed it already, chilblain lupus can also lead to systemic lupus erythematosus.

How doctors diagnose chilblain lupus

A dermatologist or a rheumatologist can diagnose chilblain lupus. Usually, healthcare providers use a test called a skin biopsy.

During a skin biopsy, your provider takes a small skin sample. They send 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.

Chilblain lupus treatment

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

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.

When should I see my healthcare provider?

If you develop red or purple, itchy or painful sores on your fingers, heels, soles of your feet or toes, see your healthcare provider. They’ll be able to diagnose your condition and get you on your way to treatment.

You may also want to ask your healthcare provider:

• What’s 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?

How can I prevent chilblain lupus?

You can reduce your chance of developing chilblain lupus by protecting yourself from cold temperatures. You may:

• Get regular physical activity 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 can I expect if I have chilblain lupus?

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

In people who developed chilblain lupus randomly (not genetically), 18% will go on to develop systemic lupus erythematosus.