Lupus Erythematosus Profundus - Causes, Symptoms, and Treatment

Introduction

The immune system shields the body from bacteria and viruses. When it senses these alien invaders, it sends out an army of combat cells to attack them. Normally, the immune system can distinguish between foreign cells and its own cells. In autoimmune diseases, the immune system misidentifies parts of the body, such as joints and skin, as foreign. It releases proteins known as autoantibodies that attack healthy cells of the body. Only one organ is targeted in some autoimmune diseases. Type 1 diabetes damages the pancreas. Other diseases, such as systemic lupus erythematosus (SLE) and lupus, can affect the whole body.

What Is Lupus Erythematosus Profundus?

Lupus erythematosus profundus (LEP) is also known as lupus erythematosus panniculitis. Panniculitis involves inflammation in the subcutaneous fat. LEP is intense inflammation of the fat that causes hardened plaques that can develop into disfiguring and dimpled areas. It may present as a separate disease or coexist with systemic or discoid lupus erythematosus. Lupus panniculitis is caused by an autoimmune reaction in the deep dermis (skin) and adipose (fat) tissue. The first stage of lupus panniculitis is active inflammation with painful nodules, followed by a second stage of subcutaneous atrophy that can be cosmetically disfiguring.

What Are the Causes of Lupus Erythematosus Profundus?

Lupus, an autoimmune disease, occurs when the immune system attacks healthy tissues in the body. Lupus can result from a combination of genetics and environment. It appears that people with a genetic predisposition to lupus can develop the disease if they come in contact with something in their environment that can cause lupus. However, the cause of lupus is, in most cases, unknown. Possible triggers are:

• Infection - Infection with bacteria or viruses can cause lupus or recur in some people.

• Medication - Lupus can be caused by certain types of blood pressure medications, antiepileptic drugs, and antibiotics. People with drug-induced lupus usually recover when the drugs are stopped. In rare cases, symptoms may persist even after the drug is stopped.

• Sunlight - Exposure to sunlight can cause lupus skin lesions and can trigger internal reactions in susceptible individuals.

What Are the Risk Factors for Lupus Erythematosus Profundus?

Factors that may elevate the risk of developing lupus erythematosus profundus are:

• Sex - Lupus erythematosus profundus is more common in women.

• Age - People of all age groups are affected by lupus erythematosus profundus. It is most often diagnosed between the ages of 20 and 60.

• Race - Lupus is most common in people who are African Americans, Hispanics, and Asian Americans.

• Pre-existing Autoimmune Condition - A person with a pre-existing autoimmune condition is more likely to develop lupus erythematosus profundus.

What Are the Symptoms of Lupus Erythematosus Profundus?

The symptoms of lupus erythematosus profundus are listed below:

• Red and inflamed plaques, nodules, and some ulcers usually affect the proximal extremities, trunk, chest, buttocks, and face.

• The lesions are tender, hard, and painful.

• The lesions heal with fat atrophy (wasting of tissue) and scars.

When to Get Help for Lupus Erythematosus Profundus?

A person is advised to seek immediate medical help if they develop the following signs and symptoms:

• An unexplained rash.

• Ongoing fever.

• Persistent fatigue.

• Skin lesions that worsen when exposed to the sun.

• Shortness of breath.

• Chest pain.

• Dry eyes.

• Headaches and memory loss.

How Is Lupus Erythematosus Profundus Diagnosed?

Diagnosis of lupus erythematosus profundus is confirmed primarily by both clinical and laboratory findings.

• Complete Blood Count - This test quantifies the total number of red blood cells, white blood cells, platelet concentration, and the amount of hemoglobin in red blood cells. Results may indicate anemia, which is common in LEP. Low platelet and white blood cell count can also occur in LEP.

• Biopsy - It is sometimes done to confirm a diagnosis of lupus erythematosus profundus affecting the skin and subcutaneous tissues.

• Antinuclear Antibody (ANA) Test - A positive test for the presence of these antibodies produced by the immune system indicates that the immune system is being stimulated. Most people with lupus test positive for her ANA, but most people with positive ANA do not have lupus. If a person is ANA positive, the doctor may recommend a more specific antibody test.

What Is the Treatment of Lupus Erythematosus Profundus?

The choice of medication for lupus erythematosus profundus is given below:

• Antimalarial Drugs - Lupus erythematosus profundus often responds to treatment with antimalarial drugs such as Hydroxychloroquine (200 milligrams once or twice a day). In some cases, a combination of antimalarial drugs (e.g., Hydroxychloroquine 200 mg and Quinacrine 100 mg daily) is effective.

• Systemic Glucocorticoid Administration - It is reserved for extensive and extremely resistant lesions. Intralesional glucocorticoids are usually ineffective and may worsen the healing process of atrophy. Success with Dapsone, Azathioprine, and Thalidomide has been reported in individual cases of LEP.

• Surgical - Surgical debridement or excision of individual lesions may be attempted when all other treatment modalities have failed, and there is significant debilitation (serious weakening and loss of energy).

Complications of Lupus Erythematosus Profundus

The complications of lupus erythematosus profundus are listed below:

• Infection - The condition and its treatment can weaken the immune system, making people with lupus more susceptible to infections.

• Cancer - Having lupus erythematosus profundus appears to increase the risk of cancer. However, the risks are small.

How to Manage Lupus Erythematosus Profundus at Home?

Some tips to manage LEP at home are listed below:

• Dehydroepiandrosterone (DHEA) - Taking health supplements containing this hormone along with conventional treatments can help reduce LEP flare-ups. DHEA can also cause acne in women.

• Fish Oil - It comprises omega-3 fatty acids, which is beneficial for people with LEP. Side effects associated with fish oil supplements include belching, nausea, and a fishy taste in the mouth.

• Avoid Sun - Ultraviolet rays can cause flares, so wear protective clothing such as hats, long-sleeved shirts, and slacks, and use sunscreen with a sun protection factor (SPF) of at least 55 every time when going outdoors.

• Exercise Regularly - Exercise helps keep bones strong, reduces the risk of heart attack, and promotes overall health.

• Avoid Smoking - Smoking increases the risk of cardiovascular disease and can exacerbate the effects of lupus on the heart and blood vessels.

Conclusion

Lupus erythematosus profundus is a rare condition that is a variant of cutaneous lupus erythematosus. It is characterized by tender, often painful, erythematous subcutaneous nodules or plaques in fatty areas of the body. It occurs but may coexist with discoid lupus erythematosus (DLE), also called deep lupus or systemic lupus erythematosus (SLE). Its diagnosis is often delayed, increasing the risk of sequelae such as atrophy and calcification. Timely intervention and treatment may delay the onset of atrophy and other complications related to this condition.