Lichen Planus Overlap Syndrome

Lichen planus overlap syndrome (LPOS) is a condition characterized by the co-occurrence of lichen planus and other autoimmune diseases, such as systemic lupus erythematosus, Sjogren’s syndrome, or dermatomyositis. The term “overlap syndrome” is used to describe the presence of two or more autoimmune diseases in the same individual.

Lichen planus is a chronic, inflammatory skin disease that affects the mucous membranes and the skin. It is characterized by the formation of flat-topped, violaceous papules and plaques, which can be itchy and painful. The cause of lichen planus is not well understood, but it is thought to be related to an immune system dysfunction.

LPOS is a relatively uncommon condition, and its precise incidence and prevalence are not well known. The diagnosis of LPOS can be challenging, as the symptoms of lichen planus and the associated autoimmune diseases can overlap, making it difficult to distinguish one condition from the other.

There are several different types of LPOS, depending on the specific autoimmune disease that is present. Some of the most common types include:

1. Lichen planus-lupus erythematosus overlap syndrome (LPLEOS): This type of LPOS occurs when a person has both lichen planus and systemic lupus erythematosus. Systemic lupus erythematosus is a chronic autoimmune disease that affects multiple organ systems, including the skin, joints, kidneys, and nervous system.
2. Lichen planus-Sjogren’s syndrome overlap syndrome (LPSS): This type of LPOS occurs when a person has both lichen planus and Sjogren’s syndrome. Sjogren’s syndrome is an autoimmune disease that affects the glands that produce tears and saliva, leading to dryness of the eyes and mouth.
3. Lichen planus-dermatomyositis overlap syndrome (LPDOS): This type of LPOS occurs when a person has both lichen planus and dermatomyositis. Dermatomyositis is a rare autoimmune disease that affects the skin and muscles, causing a distinctive rash and muscle weakness.

Causes

There is no known cause of lichen planus overlap syndrome, but several factors have been identified that may contribute to its development.

1. Genetics: There may be a genetic predisposition to developing lichen planus overlap syndrome, as it has been observed to run in families.
2. Environmental triggers: Certain environmental factors, such as exposure to chemicals, metals, and drugs, have been linked to the development of lichen planus overlap syndrome.
3. Infections: Viral infections, such as hepatitis C, have been identified as possible triggers for lichen planus overlap syndrome.
4. Stress: Emotional stress and physical stress have been shown to trigger or worsen lichen planus lesions in some people.
5. Hormonal changes: Hormonal changes, such as those that occur during menopause, pregnancy, or taking oral contraceptives, have been linked to lichen planus overlap syndrome.
6. Medications: Certain medications, such as angiotensin-converting enzyme (ACE) inhibitors, have been linked to the development of lichen planus overlap syndrome.
7. Immune system dysfunction: A dysfunction in the immune system, such as an overactive immune response or an inability to regulate immune response, may contribute to the development of lichen planus overlap syndrome.
8. Inflammation: Chronic inflammation has been linked to the development of lichen planus overlap syndrome.
9. Allergies: Allergic reactions to certain substances, such as food, medications, or chemicals, may trigger lichen planus lesions.
10. Nutritional deficiencies: A deficiency in certain nutrients, such as vitamins A, C, and D, or minerals like iron or zinc, may contribute to the development of lichen planus overlap syndrome.
11. Chronic illnesses: Chronic illnesses, such as autoimmune disorders, liver disease, or diabetes, have been linked to the development of lichen planus overlap syndrome.
12. Trauma: Physical injury or trauma to the skin, such as cuts, bruises, or burns, may trigger lichen planus lesions.
13. Exposure to ultraviolet (UV) light: Excessive exposure to UV light, either from sunlight or artificial sources, has been linked to lichen planus overlap syndrome.
14. Tobacco use: Tobacco use has been linked to the development and worsening of lichen planus lesions.
15. Alcohol consumption: Alcohol consumption has been linked to the development and worsening of lichen planus lesions.
16. Hormonal imbalances: Hormonal imbalances, such as thyroid disorders, have been linked to the development of lichen planus overlap syndrome.
17. Autoimmune disorders: Autoimmune disorders, such as systemic lupus erythematosus, scleroderma, or dermatomyositis, have been linked to lichen planus overlap syndrome.
18. Inflammatory bowel disease: Inflammatory bowel disease, such as Crohn’s disease or ulcerative colitis, has been linked to lichen planus overlap syndrome.
19. Malignancy: Malignancy, or cancer, has been linked to lichen planus overlap syndrome in some cases.

Symptoms

The following are the symptoms associated with LPO:

1. Cutaneous lichen planus: This is the most common symptom of LPO and is characterized by the appearance of flat-topped, purple-colored papules (bumps) on the skin.
2. Oral lichen planus: This is a type of lichen planus that affects the oral mucosa (the lining of the mouth) and can cause painful erosions and ulcers.
3. Mucosal lichen planus: This type of lichen planus affects the mucosal membranes, such as those in the genital area, and can cause itching, burning, and painful urination.
4. Alopecia: This is a type of hair loss that can occur in individuals with LPO and is characterized by patchy hair loss on the scalp or other areas of the body.
5. Nail changes: Individuals with LPO may develop changes in the nails, such as ridges, splitting, or thickening.
6. Pruritus: This is a type of itching that can occur in individuals with LPO and is often associated with cutaneous lichen planus.
7. Photosensitivity: Individuals with LPO may develop sensitivity to sunlight and may develop rashes or worsening of skin lesions after exposure to sunlight.
8. Arthritis: Individuals with LPO may develop joint pain, swelling, and stiffness, which can be indicative of an underlying autoimmune arthritis.
9. Gastrointestinal symptoms: Individuals with LPO may develop abdominal pain, bloating, and diarrhea, which can be indicative of an underlying autoimmune enteritis.
10. Renal involvement: Individuals with LPO may develop kidney involvement, which can manifest as proteinuria (the presence of protein in the urine), hematuria (the presence of blood in the urine), or nephrotic syndrome (a group of symptoms associated with kidney disease).
11. Neuropsychiatric involvement: Individuals with LPO may develop neuropsychiatric symptoms, such as anxiety, depression, or cognitive impairment.
12. Malignancy: Individuals with LPO have an increased risk of developing malignancies, such as lymphoma or squamous cell carcinoma.
13. Thyroid involvement: Individuals with LPO may develop thyroid involvement, which can manifest as hypothyroidism (an underactive thyroid gland) or hyperthyroidism (an overactive thyroid gland).
14. Pulmonary involvement: Individuals with LPO may develop pulmonary involvement, which can manifest as interstitial lung disease or bronchiolitis obliterans (a type of lung disease).
15. Hematologic involvement: Individuals with LPO may develop hematologic involvement, which can manifest as anemia (a decrease in red blood cells) or thrombocytopenia (a decrease in platelets).
16. Ocular involvement: Individuals with LPO may develop ocular involvement, which can manifest as conjunctivitis (inflammation of the conjunctiva) or keratoconjunctivitis sicca (dry eyes).
17. Dermatitis herpetiformis: This is a type of skin rash that can occur in individuals with LPO and is characterized by small, itchy blisters that resemble those seen in herpes simplex virus infections.

Diagnosis

Here is a list of diagnosis and tests for LPOS:

1. Physical examination: A physical examination is the first step in diagnosing LPOS. The doctor will examine the skin and mucous membranes for signs of lichen planus, such as purple or pinkish-red flat-topped bumps, and signs of another autoimmune disorder, such as joint pain or a butterfly-shaped rash across the cheeks and nose.
2. Biopsy: A biopsy is a procedure in which a small sample of skin or mucous membrane is removed for examination under a microscope. This test is important for confirming the diagnosis of LPOS and ruling out other conditions that may have similar symptoms.
3. Blood tests: Blood tests can help diagnose LPOS by measuring levels of certain antibodies or markers that are associated with autoimmune disorders. Examples of blood tests include antinuclear antibody (ANA) test, anti-dsDNA test, anti-Ro/SSA and anti-La/SSB tests, and rheumatoid factor (RF) test.
4. Skin testing: Skin testing can be used to determine if a person has an allergy to a particular substance. This test may be performed to rule out other conditions that may cause similar symptoms to LPOS.
5. Complete blood count (CBC): A CBC is a blood test that measures different components of the blood, including red blood cells, white blood cells, and platelets. This test can help diagnose LPOS by revealing any changes in the levels of these components that may be associated with autoimmune disorders.
6. 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 can indicate inflammation in the body, which is often seen in autoimmune disorders.
7. C-reactive protein (CRP) test: The CRP test is a blood test that measures the level of a protein called C-reactive protein, which is produced by the liver in response to inflammation. A high CRP level can indicate inflammation in the body and suggest an autoimmune disorder such as LPOS.
8. Complement levels: Complement is a group of proteins that help the body fight infections and remove dead or damaged cells. Low levels of complement can indicate an autoimmune disorder such as LPOS.
9. Antiphospholipid antibody test: This test measures the level of antiphospholipid antibodies in the blood. Antiphospholipid antibodies are associated with blood clotting disorders and can be seen in people with LPOS.
10. HLA typing: HLA typing is a blood test that determines the presence of specific human leukocyte antigens (HLA) that are associated with increased risk for certain autoimmune disorders, including LPOS.
11. X-rays: X-rays can be used to diagnose LPOS by revealing any changes in the bones or joints that may be associated with the condition.
12. Magnetic resonance imaging (MRI): An MRI is a non-invasive imaging test that uses a magnetic field and radio waves to create detailed images of the inside of the body. This test can help diagnose LPOS by revealing any changes

Treatment

Here is a list of treatments for LPOS:

1. Topical corticosteroids: These are creams or ointments that can be applied directly to the skin to reduce inflammation and itching.
2. Systemic corticosteroids: These are oral or injectable medications that can be taken to reduce inflammation throughout the body.
3. Topical calcineurin inhibitors: These are creams or ointments that work by blocking the activity of certain immune cells that contribute to skin inflammation.
4. Systemic immunosuppressants: These are medications that suppress the immune system and can be used to treat autoimmune disorders, including LPOS.
5. Phototherapy: This involves exposing the skin to controlled amounts of ultraviolet light, which can help to reduce inflammation and improve skin symptoms.
6. Antihistamines: These medications can be taken to reduce itching and other symptoms associated with LPOS.
7. Antimalarials: These medications, originally developed to treat malaria, can also be used to treat autoimmune disorders, including LPOS.
8. Retinoids: These are medications that are related to Vitamin A and can be used to improve skin symptoms in LPOS.
9. Systemic antibiotics: These medications can be used to treat bacterial infections that may occur in LPOS.
10. Topical antibiotics: These are creams or ointments that can be applied directly to the skin to treat bacterial infections.
11. Antidepressants: These medications can be used to treat depression, anxiety, and other mood disorders that may be associated with LPOS.
12. Pain medications: These medications can be used to relieve pain and discomfort associated with LPOS.
13. Vitamin and mineral supplements: These can be used to replenish nutrients that may be depleted in LPOS.
14. Herbal remedies: Some herbs, such as aloe vera, chamomile, and calendula, have anti-inflammatory and soothing properties and can be used to treat skin symptoms in LPOS.
15. Acupuncture: This traditional Chinese therapy involves the insertion of thin needles into the skin at specific points to promote healing and reduce symptoms.
16. Massage therapy: This involves the use of manual techniques to manipulate the soft tissues of the body and can help to reduce pain and improve circulation in LPOS.
17. Physical therapy: This involves exercises and other physical activities that can help to improve mobility and reduce pain in LPOS.
18. Occupational therapy: This involves the use of specific activities and exercises to help a person perform daily activities and maintain independence in LPOS.
19. Support groups: Joining a support group can provide a person with emotional support and practical advice for coping with LPOS.
20. Lifestyle changes: Making changes to one’s diet, exercise habits, and stress levels can help to improve symptoms and overall well-being in LPOS.

It is important to note that the best treatment for LPOS will depend on the individual and the specific symptoms they are experiencing. A healthcare provider can work with a person to determine the best treatment plan based on their specific needs and medical history.