Scleredema of Buschke

Scleredema of Buschke, also known as scleredema adultorum, is a rare connective tissue disorder that is characterized by the thickening and hardening of the skin on the upper back, neck, and shoulders. It is often associated with diabetes mellitus or an upper respiratory tract infection. This condition is benign and typically resolves on its own, although it may take several years for the skin to return to its normal texture and appearance. In this article, we will discuss the different definitions and types of scleredema of Buschke, as well as provide details on its pathogenesis, clinical features, diagnosis, and treatment.

Definition of Scleredema of Buschke

Scleredema of Buschke is a rare connective tissue disorder that is characterized by the thickening and hardening of the skin on the upper back, neck, and shoulders. It is a type of skin disorder that falls under the category of scleroderma-like diseases, which are characterized by hardening of the skin and other connective tissues.

There are three types of scleredema of Buschke based on their clinical presentation:

1. Type I: This type of scleredema is the most common and is associated with diabetes mellitus or an upper respiratory tract infection. The skin in this type is thickened and hard, and it may be slightly red or brown in color. The skin may also be slightly dimpled, giving it a “peau d’orange” appearance.
2. Type II: This type of scleredema is less common and is not associated with any underlying medical condition. The skin in this type is thickened and hard, and it may be pale or pink in color. The skin may also have a shiny appearance and be less flexible than normal skin.
3. Type III: This type of scleredema is the least common and is associated with monoclonal gammopathy, which is a type of blood disorder. The skin in this type is thickened and hard, and it may be red or brown in color. The skin may also have a shiny appearance and be less flexible than normal skin.

Causes

It is often associated with other systemic diseases and can have significant morbidity and mortality. Here are possible causes for SB:

1. Infection: SB has been reported to occur following viral infections such as upper respiratory infections and cytomegalovirus infections.
2. Hyperglycemia: SB has been linked to hyperglycemia, particularly in diabetic patients. High levels of glucose in the blood can lead to the accumulation of advanced glycation end products (AGEs), which can damage the skin and other tissues.
3. Immunologic disorders: SB has been associated with autoimmune disorders such as lupus erythematosus and scleroderma.
4. Paraproteinemia: SB can occur in the context of paraproteinemia, which refers to the presence of abnormal proteins in the blood.
5. Drug reactions: Several drugs have been reported to cause SB, including interferon, methotrexate, and penicillamine.
6. Malignancy: SB can be a paraneoplastic phenomenon, meaning that it can occur as a result of an underlying cancer.
7. Hematologic disorders: SB has been reported in patients with hematologic disorders such as multiple myeloma and Waldenstrom macroglobulinemia.
8. Endocrine disorders: SB has been associated with hypothyroidism, acromegaly, and Cushing’s syndrome.
9. Chronic kidney disease: SB has been reported in patients with chronic kidney disease, particularly those on dialysis.
10. Obesity: SB has been associated with obesity, particularly in patients with a high body mass index (BMI).
11. Trauma: SB can occur following trauma, particularly in the context of burn injuries.
12. Genetic predisposition: SB has been reported in families, suggesting a genetic predisposition.
13. Radiation therapy: SB can occur following radiation therapy, particularly in the context of head and neck cancer.
14. Occupational exposure: SB has been reported in individuals with occupational exposure to rubber products, such as gloves and balloons.
15. Liver disease: SB has been associated with liver disease, particularly in the context of cirrhosis.
16. Connective tissue disorders: SB can occur in the context of other connective tissue disorders such as rheumatoid arthritis and dermatomyositis.
17. Neurological disorders: SB has been reported in patients with neurological disorders such as multiple sclerosis.
18. Vascular disorders: SB has been associated with vascular disorders such as Raynaud’s phenomenon and peripheral artery disease.
19. Vitamin deficiencies: SB has been associated with vitamin deficiencies, particularly vitamin D deficiency.
20. Unknown causes: In some cases, no underlying cause can be identified for SB.

The pathogenesis of SB is not fully understood, but it is thought to involve inflammation, fibrosis, and deposition of mucopolysaccharides in the skin. The exact mechanisms by which the above-mentioned causes lead to SB are also not fully understood, but it is likely that they involve a combination of genetic, environmental, and immunologic factors.

Symptoms

Common symptoms of scleredema of Buschke, as well as provide a detailed explanation of the condition and its treatment options.

1. Skin thickening: One of the most prominent symptoms of scleredema of Buschke is skin thickening, which can be felt as a hard, rubbery mass beneath the skin.
2. Skin discoloration: The affected area may appear reddish-brown, yellow, or grayish in color.
3. Reduced mobility: The skin may feel stiff and less elastic, which can limit movement in the affected area.
4. Shoulder pain: Patients with scleredema of Buschke may experience pain or discomfort in the shoulders, especially when moving the arms.
5. Neck pain: The thickened skin can also cause pain and stiffness in the neck.
6. Swelling: The affected area may become swollen and puffy.
7. Numbness or tingling: Patients may experience numbness or tingling in the affected area.
8. Difficulty swallowing: In rare cases, scleredema of Buschke can cause difficulty swallowing due to thickening of the skin around the throat.
9. Difficulty breathing: If the skin thickening extends to the chest, it can cause breathing difficulties.
10. Skin tightness: The skin may feel tight and stretched, as if it is being pulled in multiple directions.
11. Skin texture changes: The affected area may feel rough or bumpy to the touch.
12. Skin folds: As the skin thickens, it may develop folds and creases.
13. Limited joint mobility: In severe cases, the skin thickening can lead to limited mobility in the joints.
14. Itching: The thickened skin may become itchy, which can be frustrating for patients.
15. Dry skin: The affected area may become dry and flaky.
16. Fatigue: Some patients with scleredema of Buschke may experience fatigue or weakness.
17. Weight loss: In rare cases, scleredema of Buschke can cause weight loss due to reduced appetite and difficulty swallowing.
18. Vision problems: In extremely rare cases, scleredema of Buschke can affect the eyes and cause vision problems.
19. Difficulty speaking: As with difficulty swallowing, thickening of the skin around the mouth and throat can make it difficult to speak.
20. Impaired hearing: Rarely, scleredema of Buschke can affect the ears and cause hearing loss.

Scleredema of Buschke is a rare condition that can be difficult to diagnose. The skin thickening can be mistaken for other conditions, such as scleroderma or morphea. In some cases, a biopsy may be necessary to confirm the diagnosis.

Diagnosis

Different diagnoses and tests for scleredema of Buschke, as well as provide an in-depth explanation of the condition and its symptoms.

Diagnoses and Tests for Scleredema of Buschke

1. Physical Examination – A physical examination by a dermatologist is usually the first step in diagnosing scleredema of Buschke. The dermatologist will examine the skin for any signs of thickening or hardening, as well as assess the patient’s overall health.
2. Biopsy – A skin biopsy involves taking a small sample of the affected skin for examination under a microscope. This can help to confirm the diagnosis of scleredema of Buschke, as well as rule out other conditions.
3. Blood Tests – Blood tests can be done to check for underlying systemic diseases, such as diabetes or autoimmune disorders, that may be causing the scleredema of Buschke.
4. Imaging Tests – Imaging tests, such as X-rays or CT scans, can be used to assess the extent of the skin thickening and check for any underlying internal abnormalities.
5. Skin Surface Temperature – The skin surface temperature can be measured using a thermography device. Scleredema of Buschke typically causes a decrease in skin temperature, which can be detected by this test.
6. Skin Elasticity – Skin elasticity can be measured using a skin suction device. Scleredema of Buschke usually causes a decrease in skin elasticity, which can be detected by this test.
7. Skin Thickness – Skin thickness can be measured using an ultrasound device. Scleredema of Buschke usually causes an increase in skin thickness, which can be detected by this test.
8. Capillaroscopy – Capillaroscopy involves using a microscope to examine the small blood vessels in the skin. This can help to identify any abnormalities that may be contributing to the scleredema of Buschke.
9. Magnetic Resonance Imaging (MRI) – MRI can be used to visualize the skin and underlying tissue, and can help to assess the extent of the skin thickening and any underlying internal abnormalities.
10. Electromyography (EMG) – EMG can be used to assess muscle function and detect any abnormalities that may be contributing to the scleredema of Buschke.
11. Nerve Conduction Velocity (NCV) – NCV can be used to assess nerve function and detect any abnormalities that may be contributing to the scleredema of Buschke.
12. Glycosylated Hemoglobin (HbA1c) – HbA1c is a blood test that measures the average blood sugar level over the past 2-3 months. This test can help to diagnose or monitor diabetes, which is a common underlying cause of scleredema of Buschke.
13. Antinuclear Antibody (ANA) Test – ANA test can help to diagnose autoimmune disorders, which are a common underlying cause of scleredema of Buschke.
14. Rheumatoid Factor (RF) Test – RF test can help to diagnose rheumatoid arthritis, which is a common underlying cause of scleredema of Buschke.
15. C-Reactive Protein (CRP) Test – CRP test can help to identify inflammation in the body, which may be contributing to the scleredema of Buschke.

Treatment

There is no cure for scleredema of Buschke, but there are several treatments that can help manage symptoms. In this article, we will discuss  treatments for scleredema of Buschke.

1. Moisturizers – Moisturizers can help alleviate dryness and tightness in the affected areas of the skin. Using a moisturizer regularly can also help prevent skin cracking, which can lead to infections.
2. Topical corticosteroids – Topical corticosteroids are anti-inflammatory drugs that can help reduce inflammation and swelling in the affected areas of the skin. They are available in different strengths and can be applied directly to the skin.
3. Immunosuppressants – Immunosuppressants are medications that suppress the immune system. They can help reduce the inflammation and swelling associated with scleredema of Buschke.
4. Skin moisturizers – Regular use of skin moisturizers can help prevent dryness and cracking of the skin in individuals with scleredema of Buschke.
5. Urea cream – Urea cream is a moisturizing cream that can be especially helpful for individuals with scleredema of Buschke. Urea is a natural compound that helps to soften and hydrate the skin.
6. Salicylic acid – Salicylic acid is a type of keratolytic agent that can help remove dead skin cells and soften thickened areas of the skin.
7. Topical retinoids – Topical retinoids are medications that are derived from vitamin A. They can help improve the texture and appearance of the skin in individuals with scleredema of Buschke.
8. Antibiotics – Antibiotics may be prescribed for individuals with scleredema of Buschke who have developed bacterial infections in the affected areas of the skin.
9. Physical therapy – Physical therapy can help improve range of motion in the affected areas of the skin. A physical therapist can teach exercises and stretches to help improve mobility.
10. Massage therapy – Massage therapy can help improve circulation and reduce swelling in the affected areas of the skin. It can also help alleviate pain and stiffness.
11. Ultraviolet light therapy – Ultraviolet light therapy, also known as phototherapy, involves exposing the affected areas of the skin to ultraviolet light. This can help reduce inflammation and improve skin elasticity.
12. Laser therapy – Laser therapy uses a high-energy beam of light to target and remove the thickened skin. It can help improve skin texture and reduce pain and stiffness.
13. Cryotherapy – Cryotherapy involves applying cold temperatures to the affected areas of the skin. This can help reduce inflammation and alleviate pain and stiffness.
14. Phototherapy – Phototherapy involves the use of ultraviolet (UV) light to treat scleredema of Buschke. This treatment works by suppressing the immune system and reducing inflammation in the skin. Phototherapy may be used alone or in combination with other treatments.
15. Extracorporeal photopheresis – Extracorporeal photopheresis is a specialized form of phototherapy that involves removing a small amount of the individual’s blood, exposing it to UV light, and then returning it to the body. This treatment has been shown to be effective in managing the symptoms of the scleredema of Buschke.
16. Heat therapy – Heat therapy involves applying warm temperatures to the affected areas of the skin. This can help improve circulation and alleviate pain and stiffness.
17. Nonsteroidal anti-inflammatory drugs (NSAIDs) – NSAIDs are medications that can help reduce pain, inflammation, and swelling in the affected areas of the skin.
18. Antihistamines – Antihistamines are medications that can help reduce itching and allergic reactions in the affected areas of the skin.
19. Colchicine – Colchicine is a medication that can help reduce inflammation and swelling in the affected areas of the skin.
20. Pentoxifylline – Pentoxifylline is a medication that can help improve circulation and reduce inflammation in the affected areas of the skin.
21. Methotrexate – Methotrexate is a medication that can help suppress the immune system and reduce inflammation in the affected areas of the skin.
22. Cyclophosphamide – Cyclophosphamide is a medication that can help suppress the immune system and reduce inflammation in the affected areas of the skin.
23. Thalidomide – Thalidomide is a medication that can help reduce inflammation and improve skin texture in the affected areas of the skin.
24. Intravenous immunoglobulin (IVIG) IVIG is a treatment that involves infusing the patient with immunoglobulin proteins obtained from human plasma. This can help reduce inflammation and suppress the immune system.

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