Chilblain lupus erythematosus (CHLE) is a rare type of cutaneous lupus erythematosus (CLE). Symptoms, such as lesions and swelling, can develop after exposure to cold temperatures.

CHLE may have a genetic cause, or it may occur spontaneously.

A doctor can recommend medications to help manage symptoms, while lifestyle changes may also help.

Read on to learn more about the symptoms and causes of CHLE. This article also looks at how doctors diagnose the condition, treatment options, possible complications, and more.

View the slideshow below for photos of chilblain lupus.

CHLE typically presents as red or dusky purple thickened or elevated areas of skin. A person may also experience pruritus, or itchiness.

Other symptoms can include:

  • ulcers
  • reduced skin pigmentation
  • Raynaud phenomenon, which occurs when the blood stops properly flowing to the fingers and toes

CHLE most commonly develops on the:

  • fingers
  • toes
  • soles of the feet
  • heels

It may also affect other parts of the body, such as the:

  • ears
  • elbows
  • lower legs
  • knees
  • knuckles
  • nose
  • palms

As CHLE is a type of CLE, it is an autoimmune condition, which occurs when something causes the immune system to mistakenly attack the body’s cells.

The exact cause of CHLE is unclear, but symptoms develop when the blood vessels spasm, or contract. This process causes a micro-injury that affects circulation.

Some genetic mutations may be responsible for developing CHLE. These include changes in the TREX1 and SAMHD genes.

Familial CHLE typically begins in childhood. Sporadic CHLE can occur later in life, with exposure to the cold being the main trigger.

Doctors use major and minor criteria to diagnose CHLE. A person must have both major criteria and at least one minor criterion to receive a diagnosis.

Both criteria contain a mix of physical symptoms and immunological markers, such as how the skin responds to cold and whether the blood contains a particular group of antibodies.

CHLE symptoms can resemble pernio, or chilblains. However, while chilblains typically do not develop into another condition, CHLE can progress to systemic lupus erythematosus (SLE), so an early and accurate diagnosis is crucial.

Treatment for CHLE can include medications and lifestyle changes.

Lifestyle changes mostly focus on protecting against the cold and may include:

  • avoiding cold and moist environments
  • keeping the home heated and insulated
  • wearing protective gloves and footwear
  • immersing hands in warm water throughout the day
  • keeping physically active to maintain body temperature

If symptoms do not respond to lifestyle changes or a person develops an infection, they may require medication. Possible medical treatments include:

In certain cases, such as severe damage to the skin tissue, a person may need a skin graft.

Possible complications of CHLE can include:

  • progression to SLE
  • skin infections
  • Koebner phenomenon, which refers to skin lesions following injury to the skin

Working with a doctor and following a suitable treatment plan for CHLE may help to reduce the likelihood of complications.

As the exact cause of CHLE is unclear, it is not possible to completely prevent the condition.

However, a person may be able to reduce the severity of a flare by keeping warm and moving regularly to maintain blood flow and body temperature.

How serious is chilblain lupus?

CHLE can be a serious condition. Without treatment, a person is at risk of repeated bacterial skin infections.

Sporadic CHLE leads to the development of SLE in around 18% of cases. However, if a person has familial CHLE, which usually begins in childhood, it is unlikely to lead to SLE.

What is the difference between chilblains and chilblain lupus?

Pernio, or chilblains, is an isolated condition that presents similarly to CHLE. The blood vessels spasm, causing symptoms. However, unlike CHLE, it is not an autoimmune condition.

A person with CHLE may develop SLE, although chilblains alone are not a risk factor for SLE.

What is the difference between lupus pernio and chilblain lupus?

Lupus pernio is a rare type of sarcoidosis, which occurs when inflammatory white blood cells enter tissue and cause granulomas to develop. Granulomas are little lumps or nodules that usually develop on the nose, ears, and cheeks.

CHLE occurs when sores and hardened skin develop on the fingers and toes. Like chilblain lupus, changes in the immune system are responsible for symptoms.

Chilblain lupus erythematosus (CHLE) is a type of cutaneous lupus erythematosus (CLE) that usually develops in response to exposure to the cold. It causes skin changes, swelling, and inflammation, typically on the fingers and toes.

To help prevent flares or reduce their severity, a person needs to stay as warm as possible.

Anti-inflammatories and other medications may be effective at managing symptoms of the condition if lifestyle changes alone do not help. A person may need to take antibiotics to treat skin infections.

People who inherit CHLE are unlikely to develop systemic lupus erythematosus (SLE). However, if CHLE spontaneously occurs later in life, this increases the likelihood of SLE.

A person can contact their doctor if they have concerns about the symptoms of CHLE or developing SLE.