Lupus Pernio | Lupus Pernio in Sarcoidosis

Lupus pernio is a skin lesion that's typically bluish-red in color. It may be non-life-threatening, but it can still affect the overall quality of your life, especially cosmetically.

Keep reading to know more about this condition and if you're at risk.

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Understanding Lupus Pernio: A Manifestation of Sarcoidosis

What Is Sarcoidosis?

Sarcoidosis is a disease that can affect the different systems of your body. It's a condition wherein there's a growth of granulomas in your body. Granulomas are nodules or lumps of white blood cells and tissues that form when there's inflammation.

What Are the Organs Involved in Sarcoidosis?

1. Lungs

Sarcoidosis can affect multiple organs in your body, but it most often affects your lungs. This is why respiratory physicians generally manage patients with this condition.

Among individuals with sarcoidosis, the lungs are affected more than 90% of the time. This disease can cause mediastinal lymphadenopathy, a condition wherein the lymph nodes in the mediastinal cavity become enlarged.

2. Liver

Sarcoidosis can cause cholestasis or a decrease in your bile flow, liver nodules, and liver cirrhosis.

3. Eyes

Eyes are affected in around 20-30% of people with sarcoidosis. It can cause optic neuritis, uveitis, and pink eye or conjunctivitis.

4. Heart

The heart is affected around 5% of the time. It can cause heart block and bundle branch block. Additionally, inflammation or granulomas can affect your cardiac muscle, which can result in heart failure.

5. Kidneys

Kidneys are affected in less than 5% of individuals with this disease. It can cause kidney disorders, such as interstitial nephritis, kidney stones, and nephrocalcinosis.

6. Central Nervous System

The central nervous system is also affected around 5-15% of the time. People with sarcoidosis might develop nodules in their brains.

7. Peripheral Nervous System

The nervous system is involved in around 5-13% of sarcoidosis cases. It can lead to facial muscle weakness or Bell's palsy, as well as mononeuritis multiplex.

8. Skin

The skin is affected in around 20-35% of individuals with this condition. Some of the usual skin changes include:

• Erythema nodosum: This is a skin inflammation characterized by tender, red or violet nodules on your shins or lower legs. This is a bit painful, especially when pressed upon.
• Lupus pernio: This is characterized by purple patches, deep lumps, or nodules on your face.

Clearly, sarcoidosis can affect your whole body. But we'll focus on the more striking manifestation of sarcoidosis on your skin–the lupus pernio (LP).

What Is Lupus Pernio?

The term “lupus pernio” is derived from Latin, which literally means “wolf” (lupus) and “chilblain” (pernio). It's a sign of sarcoidosis and is slowly progressive.

It begins with some purple lesions around your ear area and on the tip of your nose. Then, it enlarges into purple nodules on your face and even fingers.

Thankfully, LP isn't infectious.

Who Gets Lupus Pernio?

Some people are at a higher risk of developing LP. Its yearly cases range from one to 64 for every 100,000 individuals.

Here are some risk factors of LP:

• Ethnicity: Lupus pernio is more frequent and more serious among African ethnic groups compared with Caucasians.
• Age: It's more common among people between the ages of 45 and 65.
• Sex: It's twice more common in females than in males.

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What Causes LP?

Despite extensive research, the cause of LP remains uncertain.

Sarcoidosis is thought to be a result of a persisting cell-mediated immunity. CD4 T cells activate macrophages, which stimulate the formation of granulomas.

Experts think that some cases of LP may be due to an abnormal reaction of your body's immune system to infectious agents, substances, or chemicals.

Some examples of these organisms include:

• fungi
• Mycobacterium paratuberculosis
• histoplasmosis

What Are the Signs and Symptoms of LP?

Signs and symptoms of LP vary from person to person. Some may be asymptomatic, while others may have severe symptoms.

Usually, LP presents itself as red to purple nodules and plaques on your cheeks, nose, earlobes, lips, and forehead. These lumps are hardened and raised. And as scaly and itchy as it may look, LP is rarely tingling or painful.

However, cosmetic disfigurement or deformity is the most usual complaint. Eventually, as it heals, LP may develop into spider vein-like scarring and thickening of tissue. It can also cause swelling in your toes and hands.

Moreover, the skin lesions may stretch up to the bones and cartilage in your nose, which may result in nosebleeds.

How Is LP Diagnosed?

Lupus pernio may be known by its general appearance and history. But its diagnosis may be hard to establish.

Your skin's edge may be inspected using diascopy. Diascopy is a test performed by applying pressure to your skin using either a piece of clear glass slide, a finger, or a plastic. It observes color changes and shows an appearance that's typical of granulomatous inflammation.

The diagnosis may also be confirmed with lung, skin, or lymph node biopsy.

Furthermore, individuals with LP are at a greater risk for upper respiratory tract and pulmonary diseases. Because of this, the following tests may also be performed for diagnosis:

• electrocardiogram (ECG)
• chest x-ray
• pulmonary function tests
• blood tests

What Is the Treatment for Sarcoidosis and LP?

Sadly, there's no cure for sarcoidosis. It may last for a long time, and it may even cause organ damage.

But the good thing is that in most cases, granulomas heal and go away on their own. Additionally, most patients do very well even though there's no available treatment.

Meanwhile, the goal of LP treatment is to enhance the patient's general appearance and prevent the skin from scarring. The treatment is also based on the symptom severity.

Your options are as follows:

• carbon dioxide and pulsed dye laser treatment
• intralesional steroid injection
• drugs, such as methotrexate, systemic corticosteroids, infliximab, hydroxychloroquine, and adalimumab
• topical corticosteroids

On the other hand, you may also apply cosmetic camouflage, like powders, creams, and liquids, to cover skin lesions and conceal irregularities on your face or body.

Check out this video by American Lung Association to learn more about sarcoidosis, including the symptoms you may feel when you have this condition:

Lupus pernio is an indicator of sarcoidosis, an illness that can affect your entire body. Early recognition and treatment are essential to keep this condition from getting worse.

If left untreated, LP may lead to cosmetic defects, which can affect your physical and psychological well-being. So if you're experiencing any open sores, raised patches, or deep lumps, consult your doctor immediately.

Are you experiencing any signs and symptoms of lupus pernio? Have you already consulted your healthcare provider? Please share with us your experience in the comment section below!

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