What is Pernio (Chilblains)?
Pernio, also known as chilblains, is a rare condition that causes inflammation. The word “chilblains” comes from old English words – “chill” meaning cold and “blegen” meaning sore. Pernio mostly affects the skin on our limbs, like our hands or feet, and usually happens in response to cold and damp conditions.
While pernio most often occurs for no apparent reason (doctors call this idiopathic) and goes away after a while, there are also longer-lasting types. In some cases, pernio can be linked to other health problems. The most common of these is systemic lupus erythematosus which can cause a variant of pernio known as chilblain lupus erythematosus or CHLE. Because of this link, if someone has pernio, their doctor might check for other conditions that could be causing it.
Statistically, women are more likely to develop pernio than men. It’s also more common among people who live in cold climates.
Doctors mainly diagnose pernio by looking at the patients symptoms and examining them. They don’t usually need to perform a biopsy (a procedure where tissue is removed from the body for examination) as the results usually don’t provide specific information about the disease.
The best way to manage pernio is to try and prevent it from happening in the first place. This means it’s important to steer clear of the cold as much as possible. Additionally, certain medications like nifedipine and other calcium channel blockers may help to heal existing lesions. These types of drugs help to increase blood flow which can improve symptoms.
In general, people with pernio have a good outlook as it doesn’t usually cause ongoing problems.
What Causes Pernio (Chilblains)?
Pernio, a condition that affects the skin usually due to exposure to cold, is mostly believed to occur without any identifiable cause. That being said, there are a few rare forms of pernio that occur secondary to other conditions. These forms are so rare, however, that it’s been difficult to study their direct relationship due to how uncommon they are. The most frequently seen version of this secondary pernio is associated with a disease called systemic lupus erythematosus, which can further lead to the development of chilblain lupus erythematosus (CHLE).
Interestingly, research has discovered cases where pernio was the first noticeable symptom in patients who were later diagnosed with acute lymphoblastic leukemia, which is a type of blood cancer.
Risk Factors and Frequency for Pernio (Chilblains)
Pernio, a condition usually linked to cold, damp climates, tends to affect women more than men. Places without central heating, like parts of the UK and Northern Europe, commonly experience more cases. Because it’s linked to the cold, people tend to see more of it in winter, and it often comes back when the weather starts to get warmer in the spring.
Signs and Symptoms of Pernio (Chilblains)
Pernio, or “chilblains,” usually shows up as multiple red and blue skin spots – they can be flat, raised, or even small nodules. This condition generally happens as a response to being in cold, damp places. These skin spots are often symmetrical and most commonly affect both hands or feet, specifically focusing on the fingers and toes. However, it can also affect other parts of the body but less frequently. The spots typically start to show up within a day after being exposed to the cold and usually go away within a few weeks.
In some instances, the lesions can turn into blisters or ulcers, and affected individuals may experience symptoms like burning sensations, tenderness, or itchiness. In more severe cases, prolonged exposure to cold and damp conditions has even been known to cause issues with the nails.
However, there can be complications. One significant issue to be wary of is the risk of a secondary bacterial infection, especially in patients who develop blisters or ulcers due to pernio. Infections can happen whenever there’s a break in the skin, which provides an entry point for bacteria. Therefore it is particularly important to treat these patients promptly and aim for quick healing of any blisters or ulcers.
Testing for Pernio (Chilblains)
When a doctor is trying to diagnose pernio – a condition that affects your skin when it’s cold – they will start by asking you about your symptoms, when you noticed them, if you’ve been in cold conditions recently, and whether the symptoms improve when you aren’t exposed to the cold. These questions can help your doctor to narrow down whether you have pernio or a similar medical condition where the blood vessels in your skin respond unusually to the cold.
A skin biopsy, which is a test where a small sample of your skin is taken for examination, might not always be helpful in diagnosing pernio. This is because the changes seen under the microscope in the skin of people with pernio can be quite general and are seen in other conditions as well. However, certain findings such as a high number of a type of white blood cell called lymphocytes, swelling within the skin layers (dermal edema), and death of skin cells (necrotic keratinocytes) can support the diagnosis of pernio.
Additionally, your doctor might order some basic blood tests at the start of your evaluation for pernio. This might include a complete blood count to check your overall health, an antinuclear antibody test to check for autoimmune diseases, and a serum protein electrophoresis to measure the amounts of different proteins in your blood. Depending on your exact symptoms and what might have caused them, your doctor might test for cryoglobulins in your blood. Cryoglobulins are proteins that thicken or gel below body temperature and can sometimes be associated with pernio. However, because this is quite rare, it’s not usually part of the initial tests and is only considered after the results of the first tests have been reviewed.
Treatment Options for Pernio (Chilblains)
If you’re experiencing symptoms of pernio (also known as chillblains), a condition causing inflammation and irritation on extremities exposed to cold, the first step towards treatment is to avoid further cold exposure. Keeping your hands and feet warm with the help of gloves or socks can be really helpful. It’s also advisable to make some changes to your lifestyle, including quitting smoking, as it can make the symptoms worse by causing narrowing of blood vessels (vasospasm).
Once you’ve taken these initial steps, your doctor may recommend additional treatments. This could include topical creams that contain corticosteroids – a type of medication that helps to reduce inflammation. However, it’s important not to use these creams excessively, or for too long, as they can cause the skin to become thinner (a condition known as tissue atrophy).
Nifedipine, a type of medicine known as a calcium channel blocker, which widens blood vessels (vasodilatory effects), might also be helpful in treating pernio. It’s thought to help reduce inflammation and repair skin damage, and some small studies have even found that it could help prevent more symptoms from developing in the future.
What else can Pernio (Chilblains) be?
When a doctor diagnoses pernio, a skin condition caused by exposure to cold, it’s important to also consider chilblain lupus erythematosus (CHLE). CHLE is a rare skin condition that can often be mistaken for pernio or conditions related to blood vessels. A diagnosis of CHLE becomes crucial as it can suggest the presence of certain inherited genetic factors and possible connections to a condition called systemic lupus erythematosus, a long-term autoimmune disorder.
CHLE’s cause is usually unknown, but there’s a link to various genetic mutations in some families. Treating CHLE often involves using steroids to alleviate symptoms. However, research has suggested other medications, like JAK inhibitors, could also be effective. Even though the risk of someone with CHLE developing systemic lupus erythematosus is low, recommended practice still involves further testing due to the possible long-term impact of undiagnosed systemic lupus erythematosus.
The doctor should also think about other conditions especially those linked to blood vessels that constrict as part of the diagnosis process:
- Raynaud phenomenon
- Acrocyanosis
- Cryoglobulinemia
- Cold panniculitis
What to expect with Pernio (Chilblains)
The overall outlook for pernio (also known as chilblains), a condition that causes itchy inflammation on the skin usually due to cold weather, is generally very good. The skin problems caused by pernio are rarely serious and in most cases, they can be easily treated without causing any long-term complications.
For people who get pernio as a part of chronic systemic conditions like CHLE (which stands for chronic cutaneous lupus erythematosus, a skin-related autoimmune disease), managing the underlying disease well, along with treating the symptoms of pernio, can help in quickly healing the skin problems caused by pernio.
Possible Complications When Diagnosed with Pernio (Chilblains)
The complications after experiencing pernio are similar to those associated with other conditions that cause blood vessels to constrict. If the blood vessels stay constricted for a long time, it can even lead to tissue death. Besides, openings and breakdowns in the skin may occur. Both doctors and patients should keep an eye out for any signs of an infection happening.
Complications to Watch Out For:
- Blood vessels constriction
- Tissue death due to prolonged constriction
- Skin breakdown
- Openings in the skin
- Signs and symptoms of infection
Preventing Pernio (Chilblains)
Patients should be informed about how pernio sores develop and how they can best prevent outbreaks. The information provided should focus on how to avoid these sores by protecting oneself from cold weather. Wearing insulated clothing, gloves, and socks can help to prevent unexpected contact with cold environments.
