Lupus erythematosus tumidus is a chronic and benign form of CLE that is characterized by the presence of erythematous and infiltrated plaques on sun-exposed areas of the skin. The lesions are typically round or oval in shape, and they can vary in size from a few millimeters to several centimeters in diameter. The surface of the plaques is smooth and shiny, and they are often described as being “tumid” or “bulging”.
LET can be classified into two main types based on the clinical presentation of the lesions:
- Classic LET: This type of LET is characterized by the presence of erythematous and infiltrated plaques on sun-exposed areas of the skin. The lesions are typically asymptomatic or mildly pruritic, and they can persist for months or even years. Classic LET is often associated with systemic lupus erythematosus (SLE), a chronic autoimmune disease that can affect multiple organs and tissues in the body.
- Hypertrophic LET: This type of LET is characterized by the presence of erythematous and hyperkeratotic plaques on sun-exposed areas of the skin. The lesions are typically thicker and more elevated than those seen in classic LET, and they can be painful or pruritic. Hypertrophic LET is often associated with chronic discoid lupus erythematosus (DLE), a subtype of CLE that is characterized by the presence of coin-shaped lesions on the face, scalp, and ears.
Causes
The exact cause of LET is not fully understood, but it is believed to be a result of genetic, environmental, and immunological factors. In this article, we will discuss causes of LET in detail.
- Genetics: Studies have shown that LET can run in families, indicating a genetic component to the disease. Several genes have been identified that may play a role in the development of LET, including HLA-DR3 and HLA-DR4.
- Environmental factors: Exposure to sunlight and ultraviolet (UV) radiation is a known trigger for LET. Other environmental factors that may play a role in the development of LET include infections, drugs, and chemicals.
- Infections: Certain infections, such as Epstein-Barr virus (EBV), human herpesvirus 6 (HHV-6), and cytomegalovirus (CMV), have been linked to the development of LET.
- Medications: Some medications, such as thiazide diuretics, anticonvulsants, and calcium channel blockers, have been associated with the development of LET.
- Hormones: LET is more common in women than in men, and hormonal factors may play a role in the development of the disease. Studies have shown that LET may be more common during periods of hormonal fluctuation, such as during pregnancy or menopause.
- Immunological factors: LET is an autoimmune disorder, which means that the immune system attacks healthy tissues in the body. In LET, the immune system attacks the skin, causing the characteristic lesions.
- Abnormalities in the complement system: The complement system is a part of the immune system that helps to destroy foreign substances in the body. Abnormalities in the complement system have been linked to the development of LET.
- Abnormalities in cytokines: Cytokines are proteins that play a role in the immune response. Abnormalities in cytokine levels have been observed in patients with LET.
- Abnormalities in T cells: T cells are a type of immune cell that play a role in the immune response. Abnormalities in T cell function have been observed in patients with LET.
- Abnormalities in B cells: B cells are a type of immune cell that produce antibodies. Abnormalities in B cell function have been observed in patients with LET.
- Abnormalities in natural killer (NK) cells: NK cells are a type of immune cell that play a role in killing infected or cancerous cells. Abnormalities in NK cell function have been observed in patients with LET.
- Abnormalities in dendritic cells: Dendritic cells are a type of immune cell that present antigens to T cells, initiating the immune response. Abnormalities in dendritic cell function have been observed in patients with LET.
- Abnormalities in regulatory T cells (Tregs): Tregs are a type of immune cell that help to regulate the immune response, preventing autoimmunity. Abnormalities in Treg function have been observed in patients with LET.
- Abnormalities in toll-like receptors (TLRs): TLRs are proteins that play a role in recognizing foreign substances in the body, initiating the immune response. Abnormalities in TLR function have been observed in patients with LET.
Symptoms
Common symptoms of lupus erythematosus tumidus, as well as provide detailed explanations of each symptom.
- Red or pink lesions: LET is characterized by the development of red or pink, smooth, and dome-shaped lesions on the skin that are usually painless and do not itch. These lesions can occur on any part of the body, but they are most commonly found on the face, neck, chest, and upper arms.
- Photosensitivity: People with LET may experience increased sensitivity to sunlight, which can cause the skin to become red, inflamed, and itchy.
- Scaly patches: LET can cause scaly patches to develop on the skin, which can be itchy and uncomfortable.
- Fatigue: Fatigue is a common symptom of autoimmune diseases, including LET. People with LET may experience extreme tiredness and lack of energy, which can impact their daily activities.
- Joint pain: LET can cause joint pain, stiffness, and swelling, which can affect the hands, wrists, knees, and ankles.
- Headaches: Headaches are a common symptom of autoimmune diseases, including LET. People with LET may experience frequent headaches, which can range from mild to severe.
- Muscle pain: LET can cause muscle pain and weakness, which can affect the arms, legs, and back.
- Fever: People with LET may experience fever, which is a common symptom of autoimmune diseases.
- Anemia: Anemia is a condition in which there is a decrease in the number of red blood cells in the body, leading to fatigue and weakness. Anemia can be a symptom of LET.
- Raynaud’s phenomenon: Raynaud’s phenomenon is a condition in which the fingers and toes become numb, cold, and painful in response to cold temperatures or stress. It can be a symptom of LET.
- Chest pain: LET can cause chest pain, which can be a symptom of inflammation of the lining of the heart or lungs.
- Shortness of breath: Shortness of breath can be a symptom of inflammation of the lining of the heart or lungs, which can occur in LET.
- Mouth sores: LET can cause mouth sores, which can be painful and uncomfortable.
- Hair loss: Hair loss can be a symptom of autoimmune diseases, including LET. People with LET may experience thinning of the hair or bald patches.
- Swollen lymph nodes: LET can cause swollen lymph nodes, which are small, bean-shaped structures that are part of the immune system.
- Rash: LET can cause a rash on the skin, which can be red, scaly, and itchy.
- Dry eyes: LET can cause dry eyes, which can be uncomfortable and cause blurred vision.
- Dry mouth: LET can cause dry mouth, which can be uncomfortable and increase the risk of tooth decay and gum disease.
- Kidney problems: LET can cause inflammation of the kidneys, which
Diagnosis
Diagnoses:
- Clinical examination: A physical examination can reveal the presence of red, raised lesions on the skin, which are characteristic of LET.
- Skin biopsy: A skin biopsy can confirm the presence of LET by revealing the characteristic changes in the skin that occur in this condition.
- Blood tests: Blood tests can reveal the presence of autoantibodies, such as anti-Ro and anti-La, which are often present in patients with lupus.
- ANA test: An antinuclear antibody (ANA) test can detect the presence of antibodies that target the nuclei of cells, which are often present in patients with lupus.
- Anti-dsDNA test: This test can detect the presence of antibodies that target double-stranded DNA, which are often present in patients with lupus.
- ENA panel: An extractable nuclear antigen (ENA) panel can detect the presence of antibodies against a variety of nuclear antigens, which are often present in patients with lupus.
- Complement levels: Complement levels, including C3 and C4, may be decreased in patients with lupus.
- Complete blood count: A complete blood count can reveal anemia, leukopenia, or thrombocytopenia, which are common in patients with lupus.
- Urinalysis: A urinalysis can detect the presence of protein or blood in the urine, which may indicate kidney involvement in lupus.
- Renal function tests: Renal function tests can reveal the presence of kidney damage in lupus.
- Chest X-ray: A chest X-ray can reveal the presence of pleural effusions, which are a common complication of lupus.
- Echocardiogram: An echocardiogram can detect the presence of pericarditis, which is a common complication of lupus.
- Electromyography (EMG): EMG can be used to evaluate muscle weakness, which is sometimes associated with lupus.
- Magnetic resonance imaging (MRI): MRI can be used to evaluate joint pain and swelling, which are sometimes associated with lupus.
- Computerized tomography (CT) scan: A CT scan can be used to evaluate lung involvement in lupus.
- Arthroscopy: Arthroscopy can be used to evaluate joint damage in patients with lupus.
- Joint aspiration: Joint aspiration can be used to evaluate joint pain and swelling, which are sometimes associated with lupus.
- Antiphospholipid antibody test: This test can detect the presence of antiphospholipid antibodies, which are sometimes associated with lupus.
- Skin patch test: A skin patch test can detect the presence of ultraviolet light sensitivity, which is sometimes associated with lupus.
- Phototesting: Phototesting can be used to evaluate the severity of ultraviolet light sensitivity in patients with lupus.
Treatment
Treatments for lupus erythematosus tumidus.
- Topical corticosteroids: Topical corticosteroids are creams or ointments that are applied directly to the skin. They work by reducing inflammation and suppressing the immune system. Topical corticosteroids are often the first line of treatment for LET and can help reduce redness, swelling, and itching.
- Antimalarials: Antimalarials, such as hydroxychloroquine, are commonly used to treat LET. They work by reducing inflammation and suppressing the immune system. Antimalarials can also help prevent flare-ups and reduce the risk of organ damage.
- Immunosuppressants: Immunosuppressants are medications that suppress the immune system. They are often used in cases where topical corticosteroids and antimalarials are not effective. Examples of immunosuppressants used to treat LET include methotrexate, azathioprine, and mycophenolate mofetil.
- NSAIDs: Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and naproxen, can help reduce pain and inflammation associated with LET. They are often used in combination with other treatments.
- Sun protection: LET is often triggered or exacerbated by exposure to sunlight. Wearing protective clothing, such as long-sleeved shirts and wide-brimmed hats, and using sunscreen can help reduce the risk of flare-ups.
- Cool compresses: Cool compresses can help reduce redness and inflammation associated with LET. They can also help relieve itching and discomfort.
- Steroid injections: Steroid injections can be used to treat localized areas of inflammation and reduce the risk of scarring.
- Dapsone: Dapsone is an antibiotic that is sometimes used to treat LET. It works by reducing inflammation and suppressing the immune system.
- Thalidomide: Thalidomide is a medication that is sometimes used to treat LET. It works by reducing inflammation and suppressing the immune system.
- Retinoids: Retinoids, such as isotretinoin, can help reduce inflammation and prevent scarring associated with LET. They are often used in combination with other treatments.
- Tacrolimus: Tacrolimus is an immunosuppressant that is sometimes used to treat LET. It works by suppressing the immune system and reducing inflammation.
- Cyclosporine: Cyclosporine is an immunosuppressant that is sometimes used to treat LET. It works by suppressing the immune system and reducing inflammation.
- Intravenous immunoglobulin (IVIG): IVIG is a treatment that involves infusing a patient with immunoglobulin, a protein that helps fight infection and inflammation. IVIG can be used to treat severe cases of LET.
- Plasmapheresis: Plasmapheresis is a treatment that involves removing plasma from the blood and replacing it with other fluids. It can be used to treat severe cases of LET.
- UVB phototherapy: UVB phototherapy involves exposing the skin to UVB light in order to reduce inflammation and suppress the immune system.
References
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