Lupus panniculitis, also known as lupus profundus, is a subtype of cutaneous lupus erythematosus (CLE) that affects the subcutaneous fat layer. It is a rare autoimmune disease that occurs when the body’s immune system attacks its own tissues, causing inflammation and damage.
There are two main types of lupus panniculitis:
- DLE (discoid lupus erythematosus) panniculitis: This type of lupus panniculitis is characterized by the development of deep, firm, and usually painful nodules that are red or purple in color. The nodules are usually found on the legs, arms, or face and can lead to scarring and permanent skin changes if left untreated.
- SLE (systemic lupus erythematosus) panniculitis: This type of lupus panniculitis is associated with systemic lupus erythematosus, a chronic autoimmune disease that can affect multiple organs and tissues. SLE panniculitis can present as nodules, plaques, or ulcers, and is often accompanied by other symptoms of SLE, such as joint pain and fatigue.
The exact cause of lupus panniculitis is not known, but it is thought to be related to a combination of genetic, environmental, and immune system factors. Here are some of the possible causes of lupus panniculitis:
- Genetic predisposition: Some studies have shown that certain genetic mutations may increase the risk of developing lupus panniculitis. For example, certain human leukocyte antigen (HLA) types have been associated with an increased risk of CLE.
- Environmental triggers: Certain environmental factors, such as exposure to UV light, infections, medications, and stress, may trigger lupus panniculitis in individuals who are genetically predisposed to the condition.
- Immune system dysfunction: Lupus panniculitis is thought to be related to a dysfunction in the immune system, which causes it to attack healthy tissue. This is why the condition is classified as an autoimmune disease.
- Hormonal factors: Hormonal changes, such as those that occur during pregnancy or menopause, may trigger lupus panniculitis in some individuals.
- Inflammatory cytokines: Inflammatory cytokines, such as tumor necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6), are involved in the development of lupus panniculitis. These cytokines play a role in the regulation of the immune system and the inflammation that is associated with the condition.
- Microbes and infections: Certain microbes, such as bacteria, viruses, and fungi, may trigger lupus panniculitis. Additionally, infections, such as streptococcal infections, may also trigger the condition.
- Nutritional deficiencies: Nutritional deficiencies, such as a lack of vitamin D, may play a role in the development of lupus panniculitis.
- Trauma: Physical trauma, such as a cut or a burn, may trigger lupus panniculitis in some individuals.
- Medications: Certain medications, such as hydralazine and procainamide, have been associated with the development of lupus panniculitis.
It is important to note that not all individuals with lupus panniculitis will have the same cause, and in many cases, the cause may not be known. Additionally, the cause of lupus panniculitis may be different for each individual, and multiple factors may contribute to the development of the condition.
The following are some of the common symptoms associated with lupus panniculitis:
- Skin nodules: The most distinctive symptom of lupus panniculitis is the development of skin nodules. These nodules can appear anywhere on the body, but they are most commonly found on the legs and arms. They are usually firm, raised, and often have a red or purplish appearance.
- Inflammation: Inflammation is a hallmark of lupus panniculitis and it can cause redness, swelling, and tenderness in the affected areas. In severe cases, the skin may become thickened and scarred.
- Rashes: Rashes are also a common symptom of lupus panniculitis, and they can appear anywhere on the body. They may be itchy, scaly, or have a purplish appearance.
- Fatigue: Many people with lupus panniculitis experience fatigue, which can be debilitating and interfere with daily activities.
- Joint pain: Joint pain and stiffness are also common symptoms of lupus panniculitis, especially in the hands, feet, and knees.
- Muscle weakness: Some people with lupus panniculitis may experience muscle weakness, which can make it difficult to perform everyday tasks.
- Raynaud’s phenomenon: Raynaud’s phenomenon is a condition in which the blood vessels in the fingers and toes constrict in response to cold temperatures or stress. This can cause the fingers and toes to turn white or blue, and can be painful.
- Photosensitivity: Many people with lupus panniculitis are sensitive to sunlight and may develop a rash or other skin reactions after exposure to UV light.
- Systemic symptoms: In some cases, lupus panniculitis can affect other parts of the body and cause systemic symptoms, such as fever, weight loss, and anemia.
- Mental symptoms: Some people with lupus panniculitis may experience depression, anxiety, and other mental symptoms as a result of the condition.
It is important to note that not all people with lupus panniculitis will experience all of these symptoms, and the severity of symptoms can vary greatly from person to person.
Diagnosis of lupus panniculitis typically involves a combination of clinical examination, medical history, and laboratory tests. Here are some of the most common diagnostic tests and procedures used to diagnose lupus panniculitis:
- Clinical examination: A physical examination is performed by a doctor to assess the symptoms and signs of lupus panniculitis. This includes looking at the skin for any red, raised, or scaly patches, and examining any areas of swelling or tenderness.
- Medical history: The doctor will also take a detailed medical history, including any past or current medical conditions, medications, and family history of autoimmune diseases.
- Skin biopsy: A skin biopsy is usually performed to confirm a diagnosis of lupus panniculitis. During the procedure, a small sample of skin is taken and examined under a microscope for signs of inflammation and other characteristic features of lupus panniculitis.
- Blood tests: Blood tests can be used to help diagnose lupus panniculitis and rule out other conditions with similar symptoms. These tests may include complete blood count (CBC), erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA) test, and anti-dsDNA test.
- Imaging tests: Imaging tests, such as ultrasound or MRI, may be ordered to assess any swelling or tenderness in the affected areas and to rule out other conditions with similar symptoms.
- Laboratory tests: The following laboratory tests may be performed to help diagnose lupus panniculitis:
a. Antinuclear antibody (ANA) test: This test looks for antibodies in the blood that are associated with autoimmune diseases, such as lupus.
b. Anti-dsDNA test: This test looks for antibodies that are specific to systemic lupus erythematosus (SLE).
c. Complement levels: This test measures the levels of certain proteins in the blood that are involved in the immune response. Low levels of complement can indicate lupus.
d. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) test: These tests measure inflammation in the body. Elevated levels can indicate the presence of an autoimmune disease.
e. Lupus anticoagulant test: This test looks for antibodies that interfere with blood clotting, which is a common complication of lupus.
f. Urine analysis: This test looks for any signs of kidney damage, which can occur in people with lupus.
g. Chest x-ray: This test can help to determine if there is any involvement of the lungs in lupus.
h. Skin biopsy: This test involves removing a small sample of skin for examination under a microscope. It can help confirm the diagnosis of lupus panniculitis and rule out other potential causes of skin nodules or ulcers.
- Other tests: In some cases, additional tests may be necessary to diagnose lupus panniculitis and rule out other conditions, such as rheumatoid arthritis or scleroderma. These tests may include skin patch testing, skin culture, or a referral to a specialist for further evaluation.
It is important to note that a diagnosis of lupus panniculitis is based on a combination of symptoms, physical examination findings, and laboratory and imaging test results. There is no single test that can definitively diagnose the condition, and a diagnosis may take some time to be confirmed.
The treatment of lupus panniculitis can be challenging and depends on the severity and extent of the disease, as well as the presence of other autoimmune conditions. The main goal of treatment is to control inflammation, prevent scarring, and improve the overall quality of life for the patient.
The following are the treatments that are commonly used for lupus panniculitis:
- Corticosteroids: Corticosteroids are the first-line treatment for lupus panniculitis. They are powerful anti-inflammatory drugs that can quickly reduce inflammation and swelling in the skin. Commonly used corticosteroids include prednisone and methylprednisolone. Corticosteroids can be administered orally, topically, or intravenously, depending on the severity of the disease.
- Immunosuppressive drugs: In cases where corticosteroids are not effective, or if the disease is severe, immunosuppressive drugs may be used. These drugs suppress the immune system and can help prevent the body from attacking its own tissues. Commonly used immunosuppressive drugs include methotrexate, azathioprine, and cyclophosphamide.
- Biologic agents: Biologic agents, such as tumor necrosis factor (TNF) inhibitors, are newer drugs that have been found to be effective in treating lupus panniculitis. TNF inhibitors, such as infliximab, work by blocking the action of TNF, a molecule that is involved in the inflammation process.
- Phototherapy: Phototherapy, or light therapy, involves exposing the skin to ultraviolet (UV) light. UV light can help reduce inflammation and prevent scarring. Phototherapy can be used alone or in combination with other treatments, such as corticosteroids or immunosuppressive drugs.
- Topical treatments: Topical treatments, such as creams, gels, and ointments, can be used to help reduce inflammation and prevent scarring in mild cases of lupus panniculitis. Commonly used topical treatments include corticosteroid creams and calcineurin inhibitors, such as tacrolimus and pimecrolimus.
- Cyclophosphamide: Cyclophosphamide is a powerful immunosuppressant drug that has been used in the treatment of lupus panniculitis.
- Mycophenolate Mofetil: Mycophenolate mofetil is an immunosuppressant drug that has been used in the treatment of lupus panniculitis.
- Intralesional Corticosteroids: Intralesional corticosteroids can be injected directly into the affected area to reduce inflammation and promote healing.
- Cryotherapy: Cryotherapy involves the use of cold temperatures to reduce inflammation and promote healing in lupus panniculitis.
- Excimer Laser Therapy: Excimer laser therapy uses ultraviolet light to target affected areas and reduce inflammation in lupus panniculitis.
- Psoralen plus Ultraviolet A (PUVA) Therapy: PUVA therapy involves the oral administration of psoralen and exposure to ultraviolet A light to reduce inflammation in lupus panniculitis.
- Narrow-Band Ultraviolet B (NB-UVB) Therapy: NB-UVB therapy involves exposure to a specific range of ultraviolet B light to reduce inflammation in lupus panniculitis.
- Surgery: In severe cases of lupus panniculitis, surgery may be necessary to remove affected tissue and prevent scarring. However, surgery is usually a last resort and is only used in cases where other treatments have not been effective.
In addition to these treatments, it is important for patients with lupus panniculitis to maintain a healthy lifestyle, including a balanced diet and regular physical activity. Stress management and adequate sleep are also important for managing the symptoms of lupus panniculitis.