Acute Cutaneous Lupus Erythematosus

Acute cutaneous lupus erythematosus (ACLE) is a type of lupus that affects the skin. Lupus is an autoimmune disease in which the body’s immune system attacks its own tissues and organs. ACLE is characterized by the development of skin lesions that appear suddenly and typically last for several weeks. These lesions are typically red, raised, and often have a scaly appearance. In this article, we will discuss the definitions and types of ACLE in detail.

  1. Definitions:

a) Lupus erythematosus (LE) is a chronic autoimmune disorder that affects various organs and tissues, including the skin, joints, kidneys, and central nervous system. There are three main types of lupus: systemic lupus erythematosus (SLE), discoid lupus erythematosus (DLE), and subacute cutaneous lupus erythematosus (SCLE). ACLE is a subtype of SLE.

b) Acute cutaneous lupus erythematosus (ACLE) is a type of lupus that primarily affects the skin. It is characterized by the development of skin lesions that appear suddenly and typically last for several weeks. ACLE is a subtype of SLE and is often accompanied by other symptoms such as fever, fatigue, and joint pain.

  1. Types:

a) Malar rash: A malar rash, also known as a butterfly rash, is a characteristic feature of ACLE. It is a red, butterfly-shaped rash that typically appears on the cheeks and nose. The rash can also extend to the forehead and chin. The rash is usually flat or slightly raised, and may be itchy or painful.

b) Photosensitivity: Photosensitivity is a common symptom of ACLE. It is an abnormal reaction of the skin to sunlight or other sources of ultraviolet radiation. Photosensitivity can cause a rash, hives, or other skin reactions. In ACLE, photosensitivity can cause the development of new lesions or the worsening of existing lesions.

c) Oral ulcers: Oral ulcers are another common symptom of ACLE. They are small, painful sores that develop in the mouth. Oral ulcers can be a sign of SLE and may also be present in other forms of lupus.

d) Non-scarring alopecia: Non-scarring alopecia is a type of hair loss that occurs in ACLE. It is characterized by the sudden onset of hair loss that is not accompanied by scarring or inflammation. The hair loss typically occurs in patches and can be temporary or permanent.

e) Bullous lupus erythematosus: Bullous lupus erythematosus (BLE) is a rare subtype of ACLE that is characterized by the development of large, fluid-filled blisters on the skin. BLE is often accompanied by other symptoms of SLE, such as fever, fatigue, and joint pain.

f) Vasculitis: Vasculitis is a rare complication of ACLE. It is an inflammation of the blood vessels that can cause damage to the skin, kidneys, and other organs. Vasculitis can cause a range of symptoms, including skin rash, joint pain, and kidney problems.

Causes

Potential causes of ACLE in detail.

  1. Genetic Factors: Research has shown that certain genetic factors can make an individual more susceptible to developing ACLE. There are several genes that have been identified as potential risk factors, including HLA-DR2 and HLA-DR3.
  2. Ultraviolet Radiation: Exposure to ultraviolet (UV) radiation from the sun or other sources can trigger ACLE in some individuals. UV radiation can cause damage to the skin and can also affect the immune system, which can lead to the development of ACLE.
  3. Medications: Certain medications can trigger ACLE in some individuals. These include medications used to treat high blood pressure, heart disease, and seizures. Additionally, some antibiotics and anti-inflammatory drugs can also trigger ACLE.
  4. Hormonal Changes: Hormonal changes, such as those that occur during pregnancy or menopause, can trigger ACLE in some individuals. This is because hormones can affect the immune system, which can lead to the development of ACLE.
  5. Infections: Certain viral and bacterial infections can trigger ACLE in some individuals. These include infections caused by the Epstein-Barr virus, cytomegalovirus, and hepatitis C virus.
  6. Stress: Stress can trigger ACLE in some individuals. This is because stress can affect the immune system, which can lead to the development of ACLE.
  7. Smoking: Smoking can trigger ACLE in some individuals. This is because smoking can cause damage to the skin and can also affect the immune system, which can lead to the development of ACLE.
  8. Chemicals: Exposure to certain chemicals, such as certain types of solvents and pesticides, can trigger ACLE in some individuals. This is because these chemicals can cause damage to the skin and can also affect the immune system.
  9. Alcohol: Alcohol can trigger ACLE in some individuals. This is because alcohol can affect the immune system, which can lead to the development of ACLE.
  10. Allergies: Allergies to certain foods or medications can trigger ACLE in some individuals. This is because allergies can affect the immune system, which can lead to the development of ACLE.
  11. Physical Trauma: Physical trauma, such as a burn or injury to the skin, can trigger ACLE in some individuals. This is because physical trauma can cause damage to the skin and can also affect the immune system.
  12. Chronic Illness: Chronic illnesses, such as diabetes and rheumatoid arthritis, can trigger ACLE in some individuals. This is because chronic illnesses can affect the immune system, which can lead to the development of ACLE.
  13. Pregnancy: Pregnancy can trigger ACLE in some individuals. This is because the hormonal changes that occur during pregnancy can affect the immune system, which can lead to the development of ACLE.
  14. Lupus Nephritis: Lupus nephritis, which is a complication of lupus that affects the kidneys, can trigger ACLE in some individuals. This is because lupus nephritis can affect the immune system, which can lead to the development of ACLE.
  15. Vaccines: Certain vaccines, such as the hepatitis B vaccine, can trigger ACLE in some individuals. This is because vaccines can affect the immune system, which can lead to the development of ACLE

Symptoms

Symptoms of ACLE in detail.

  1. Malar Rash: A malar rash, also known as a butterfly rash, is a characteristic symptom of ACLE. It is a red, flat or raised rash that appears on the cheeks and bridge of the nose, resembling the shape of a butterfly. It is often the first sign of lupus and can be a useful indicator for diagnosis.
  2. Photosensitivity: Photosensitivity is another common symptom of ACLE. It is an increased sensitivity to sunlight or artificial light that can cause a rash or other skin reactions. People with ACLE may develop rashes, hives, or blisters on sun-exposed areas, such as the face, neck, and arms.
  3. Oral Ulcers: Oral ulcers, also known as mouth sores or canker sores, are a common symptom of ACLE. They are painful, round or oval-shaped ulcers that appear inside the mouth. Oral ulcers can also appear on the lips and tongue and can cause discomfort while eating, speaking, or brushing teeth.
  4. Joint Pain and Swelling: Joint pain and swelling are common symptoms of ACLE. They can affect any joint in the body and can cause stiffness, tenderness, and limited mobility. Joint pain and swelling can be mild or severe and can affect one or many joints.
  5. Fatigue: Fatigue is a common symptom of ACLE. It is a feeling of exhaustion or lack of energy that can be persistent and debilitating. Fatigue can be caused by inflammation, anemia, or the stress of dealing with a chronic illness.
  6. Fever: Fever is a symptom of ACLE that can be caused by inflammation and autoimmune activity. It is a temporary increase in body temperature that can be accompanied by chills, sweating, and muscle aches.
  7. Headaches: Headaches are a common symptom of ACLE. They can be mild or severe and can be caused by inflammation, stress, or medications used to treat lupus.
  8. Raynaud’s Phenomenon: Raynaud’s phenomenon is a condition in which the blood vessels in the fingers and toes constrict in response to cold or stress, causing them to turn white or blue. It can be a symptom of ACLE and can cause pain, numbness, and tingling.
  9. Chest Pain: Chest pain is a symptom of ACLE that can be caused by inflammation in the lining of the heart or lungs. It can also be caused by anxiety or stress related to living with a chronic illness.
  10. Hair Loss: Hair loss is a common symptom of ACLE. It can be caused by inflammation in the scalp or by medications used to treat lupus. Hair loss can be temporary or permanent and can affect any part of the scalp.
  11. Dry Eyes: Dry eyes are a common symptom of ACLE. They can be caused by inflammation in the tear glands or by medications used to treat lupus. Dry eyes can cause discomfort, redness, and a gritty feeling in the eyes.
  12. Skin Lesions: Skin lesions are a common symptom of ACLE. They can appear as raised or flat red or purple patches on the skin. Skin lesions can also appear as small, fluid-filled blisters or as scaly patches. They can be itchy or painful and can affect any part of the body.

Diagnosis

most common diagnoses and tests used in the evaluation of ACLE.

  1. Physical examination: The first step in the diagnosis of ACLE involves a comprehensive physical examination by a physician. The physician will examine the skin for any rashes, ulcers, or lesions, as well as evaluate the joints for any signs of inflammation.
  2. ANA test: The antinuclear antibody (ANA) test is a blood test used to detect the presence of antibodies that attack the cell nucleus, a common finding in people with lupus. A positive ANA test alone is not sufficient for a diagnosis of lupus, but it can be an indicator that further testing is needed.
  3. Blood Tests: Blood tests can help confirm the diagnosis of ACLE by detecting specific antibodies associated with SLE, such as anti-nuclear antibodies (ANA), anti-dsDNA antibodies, anti-Smith antibodies, and anti-Ro/La antibodies.
  4. Complete Blood Count (CBC): A CBC measures the number of red and white blood cells and platelets in the blood. Anemia and low platelet count can be seen in patients with SLE.
  5. Erythrocyte Sedimentation Rate (ESR): An ESR test measures the rate at which red blood cells settle in a test tube over a certain period. Elevated ESR levels are a sign of inflammation, which is commonly seen in patients with SLE.
  6. C-Reactive Protein (CRP): A CRP test measures the level of C-reactive protein in the blood, which is another marker of inflammation.
  7. Complement Levels: Complement levels can be low in patients with SLE due to the consumption of complement proteins during the immune response.
  8. Antiphospholipid Antibodies: Antiphospholipid antibodies are antibodies that attack phospholipids, which are essential components of cell membranes. These antibodies can cause blood clots and increase the risk of stroke, heart attack, and other vascular events in patients with SLE.
  9. Rheumatoid Factor (RF): Rheumatoid factor is an antibody that can be elevated in patients with SLE and other autoimmune diseases.
  10. Anti-Cardiolipin Antibodies: Anti-cardiolipin antibodies are antibodies that attack cardiolipin, a component of cell membranes. Elevated levels of anti-cardiolipin antibodies can increase the risk of blood clots in patients with SLE.
  11. Anti-beta-2 Glycoprotein 1 Antibodies: Anti-beta-2 glycoprotein 1 antibodies are antibodies that attack beta-2 glycoprotein 1, a protein that binds to phospholipids on cell membranes. Elevated levels of these antibodies can increase the risk of blood clots in patients with SLE.
  12. Anti-SSA/Ro Antibodies: Anti-SSA/Ro antibodies are antibodies that attack SSA/Ro proteins, which are essential for the proper functioning of cells in the body. Elevated levels of these antibodies can cause skin rashes and other symptoms in patients with SLE.
  13. Anti-Ro/La antibodies: These antibodies are specific to lupus and can be detected by blood tests. The presence of these antibodies is associated with a higher risk of neonatal lupus in pregnant women.
  14. Complement levels: Complement is a group of proteins that play a role in the immune system. A low complement level is a common finding in people with lupus and can be detected by blood tests.
  15. Skin biopsy: A skin biopsy involves the removal of a small piece of skin tissue for examination under a microscope. A skin biopsy can help confirm the diagnosis of ACLE by revealing characteristic changes in the skin.
  16. Direct immunofluorescence (DIF): DIF is a laboratory test that involves staining a skin biopsy with a fluorescent dye to detect the presence of immune complexes in the skin. A positive DIF result can confirm the diagnosis of ACLE.
  17. Complete blood count (CBC): The CBC is a blood test that provides information about the number and type of blood cells. People with lupus often have low red blood cell counts, low platelet counts, and elevated white blood cell counts.
  18. Erythrocyte sedimentation rate (ESR): The ESR is a blood test that measures the rate at which red blood cells settle to the bottom of a tube. A high ESR is a non-specific marker of inflammation and can be elevated in people with lupus.
  19. C-reactive protein (CRP): The CRP is a blood test that measures a protein produced by the liver in response to inflammation. Elevated CRP levels can be a marker of disease activity in people with lupus.
  20. Urinalysis: A urinalysis is a test that examines the urine for the presence of abnormal substances, such as protein and blood. Proteinuria (the presence of protein in the urine) is a common finding in people with lupus and can be a sign of kidney involvement.
  21. Creatinine clearance: Creatinine is a waste product produced by muscles that is normally filtered out of the blood by the kidneys. Creatinine clearance is a measure of how well the kidneys are functioning. People with lupus can develop lupus nephritis, a condition in which the kidneys become inflamed and damaged.
  22. Kidney biopsy: A kidney biopsy involves the removal of a small piece of kidney tissue for examination under a microscope. A kidney biopsy can help confirm the diagnosis of lupus nephritis and determine the extent of the damage.
  23. Anti-dsDNA antibodies: These antibodies are specific to lupus and can be detected by

Treatment

Here are 20 treatments commonly used for ACLE, along with a detailed explanation of each one:

  1. Topical corticosteroids – These are creams or ointments containing steroids that can help reduce inflammation and itching. They are applied directly to the affected skin.
  2. Oral corticosteroids – If topical corticosteroids aren’t effective enough, oral steroids may be prescribed. These are taken in pill form and can have more systemic side effects than topical steroids.
  3. Antimalarials – These are drugs originally developed to treat malaria, but have also been found to be effective in treating lupus. The most commonly used antimalarial for ACLE is hydroxychloroquine.
  4. Nonsteroidal anti-inflammatory drugs (NSAIDs) – These are painkillers that can help relieve joint pain and reduce inflammation. Examples include ibuprofen and naproxen.
  5. Immunosuppressants – These drugs suppress the immune system to prevent it from attacking healthy tissues. Examples include azathioprine and mycophenolate mofetil.
  6. Intravenous immunoglobulin (IVIG) – This is a treatment that involves infusing immunoglobulin (antibodies) from donated blood into the patient’s bloodstream. It can help reduce inflammation and improve symptoms.
  7. Rituximab – This is a monoclonal antibody that targets B cells, a type of immune cell that is involved in the development of lupus. It can help reduce inflammation and prevent flare-ups.
  8. Belimumab – This is a monoclonal antibody that targets a protein called B-lymphocyte stimulator (BLyS) that is involved in the development of lupus. It can help reduce inflammation and prevent flare-ups.
  9. Cyclophosphamide – This is a chemotherapy drug that can suppress the immune system and reduce inflammation. It is typically used for severe cases of ACLE that are not responding to other treatments.
  10. Plasmapheresis – This is a treatment that involves removing plasma (the liquid part of the blood) from the patient’s bloodstream and replacing it with donor plasma. It can help remove antibodies that are causing inflammation.
  11. Extracorporeal photopheresis (ECP) – This is a treatment that involves removing white blood cells from the patient’s bloodstream, treating them with a photosensitizing agent, and then exposing them to ultraviolet light before returning them to the patient’s bloodstream. It can help suppress the immune system and reduce inflammation.
  12. Topical calcineurin inhibitors – These are creams or ointments that can help reduce inflammation and itching. They work by inhibiting a protein called calcineurin, which is involved in the immune response.
  13. Tacrolimus ointment – This is a topical immunosuppressant that can help reduce inflammation and itching. It works by suppressing the immune response in the affected area.
  14. Pimecrolimus cream – This is another topical immunosuppressant that can help reduce inflammation and itching. It works by inhibiting the activity of certain immune cells.
  15. Calcineurin inhibitors: These medications work by suppressing the immune system and reducing inflammation. They can be applied topically or taken orally. Calcineurin inhibitors can cause side effects such as burning or itching at the application site.
  16. Immunosuppressants: These medications are used to suppress the immune system and reduce inflammation. They can be taken orally or injected into the affected area. Immunosuppressants can cause side effects such as increased risk of infection and liver damage.
  17. Vitamin D supplements: Vitamin D can help regulate the immune system and reduce inflammation. Taking vitamin D supplements may help reduce the symptoms of ACLE. However, high doses of vitamin D can cause side effects such as nausea, vomiting, and kidney damage.
  18. Sun protection: Exposure to sunlight can trigger the symptoms of ACLE. Wearing protective clothing and using sunscreen can help reduce the risk of flare-ups.
  19. Avoiding triggers: Certain factors, such as stress and certain medications, can trigger the symptoms of ACLE. Avoiding these triggers can help prevent flare-ups.
  20. Cool compresses: Applying cool compresses to the affected area can help reduce inflammation and relieve itching.
  21. Moisturizers: Using moisturizers can help reduce dryness and relieve itching.
  22. Oatmeal baths: Adding oatmeal to a warm bath can help soothe the skin and relieve itching.
  23. Aloe vera: Applying aloe vera gel to the affected area can help reduce inflammation and relieve itching.
  24. Turmeric: Consuming turmeric may help reduce inflammation and relieve pain.
  25. Omega-3 fatty acids: Consuming foods rich in omega-3 fatty acids, such as salmon and flaxseed, may help reduce inflammation.
  26. Exercise: Regular exercise can help reduce inflammation and improve overall health.
  27. Relaxation techniques: Practicing relaxation techniques, such as meditation and yoga, can help reduce stress and prevent
References