Scleredema adultorum is a rare connective tissue disorder that primarily affects the skin, causing hardening and thickening of the skin in affected areas. The condition is characterized by diffuse, non-pitting swelling, and thickening of the skin, typically starting in the neck, shoulders, and upper back, and gradually spreading to other parts of the body. Here are the definitions and types of Scleredema adultorum along with further details:
- Scleredema adultorum of Buschke
Scleredema adultorum of Buschke, also known as scleredema adultorum type 1, is the most common subtype of the disorder. It is typically associated with a preceding streptococcal infection, and it usually develops in middle-aged adults. The condition is characterized by symmetric, non-pitting skin thickening and hardening, which starts in the neck, shoulders, and upper back and gradually spreads to other parts of the body. The skin in affected areas may appear yellowish or brownish, and it may feel tight and stiff. The underlying muscles and joints are usually unaffected, and the condition generally resolves spontaneously within 1-2 years.
- Scleredema adultorum of Buschke with diabetes mellitus
Scleredema adultorum of Buschke with diabetes mellitus, also known as scleredema diabeticorum, is a subtype of the disorder that is associated with diabetes mellitus. It typically occurs in patients with poorly controlled diabetes, and it is characterized by symmetric, non-pitting skin thickening and hardening, which usually starts in the neck, shoulders, and upper back and gradually spreads to other parts of the body. The skin in affected areas may appear yellowish or brownish, and it may feel tight and stiff. The condition may be more severe and persistent in patients with diabetes, and it may increase the risk of skin infections and other complications.
- Scleredema adultorum of Buschke with monoclonal gammopathy
Scleredema adultorum of Buschke with monoclonal gammopathy, also known as scleredema paraproteinemicum, is a subtype of the disorder that is associated with monoclonal gammopathy. It typically occurs in patients with multiple myeloma or other plasma cell dyscrasias, and it is characterized by symmetric, non-pitting skin thickening and hardening, which usually starts in the neck, shoulders, and upper back and gradually spreads to other parts of the body. The skin in affected areas may appear yellowish or brownish, and it may feel tight and stiff. The condition may be more severe and persistent in patients with monoclonal gammopathy, and it may be associated with other systemic symptoms such as fatigue, weight loss, and bone pain.
- Scleredema adultorum of Buschke associated with infections
Scleredema adultorum of Buschke associated with infections is a subtype of the disorder that is associated with various infections, including streptococcal infections, viral infections, and bacterial infections. It is characterized by symmetric, non-pitting skin thickening and hardening, which usually starts in the neck, shoulders, and upper back and gradually spreads to other parts of the body. The skin in affected areas may appear yellowish or brownish, and it may feel tight and stiff. The condition may be more severe and persistent in patients with underlying infections, and it may increase the risk of skin infections and other complications.
- Scleredema adultorum of Buschke associated with hematologic disorders
Scleredema adultorum of Buschke associated with hematologic disorders is a subtype of the disorder that is associated with various hematologic disorders, including leukemia, lymphoma
Causes
potential causes and associated details:
- Diabetes mellitus: Diabetes mellitus is a common cause of Scleredema adultorum, and the condition is more prevalent in individuals with poorly controlled diabetes. It is thought that elevated levels of blood glucose lead to glycosylation of collagen fibers, causing them to become stiffer and less pliable. This can result in thickening and hardening of the skin.
- Streptococcal infections: Several studies have reported an association between Scleredema adultorum and previous streptococcal infections. Streptococcal infections can lead to an immune response that targets the connective tissue, causing inflammation and thickening of the skin.
- Myeloma: Scleredema adultorum has been reported in individuals with multiple myeloma, a type of cancer that affects plasma cells in the bone marrow. It is thought that the abnormal production of immunoglobulins by myeloma cells can lead to connective tissue damage and the development of Scleredema adultorum.
- Paraproteinemia: Paraproteinemia is a condition characterized by the presence of abnormal proteins in the blood. Scleredema adultorum has been reported in individuals with paraproteinemia, and it is thought that the abnormal proteins can lead to connective tissue damage and thickening of the skin.
- Infections: Other infections besides streptococcal infections have been reported to cause Scleredema adultorum, including HIV, hepatitis C, and Epstein-Barr virus. In these cases, it is thought that the infection triggers an immune response that targets the connective tissue.
- Autoimmune diseases: Scleredema adultorum has been reported in individuals with autoimmune diseases, such as lupus and rheumatoid arthritis. In these cases, it is thought that the immune system attacks the connective tissue, leading to thickening and hardening of the skin.
- Alcohol abuse: Alcohol abuse has been reported as a potential cause of Scleredema adultorum. It is thought that the toxic effects of alcohol on the connective tissue can lead to thickening and hardening of the skin.
- Obesity: Obesity has been reported as a risk factor for Scleredema adultorum, particularly in individuals with poorly controlled diabetes. It is thought that the excess weight and associated metabolic changes can lead to connective tissue damage and the development of Scleredema adultorum.
- Hyperlipidemia: Hyperlipidemia, or high levels of lipids in the blood, has been reported as a potential cause of Scleredema adultorum. It is thought that the excess lipids can lead to connective tissue damage and the development of the condition.
- Radiation therapy: Radiation therapy has been reported to cause Scleredema adultorum in rare cases. It is thought that the radiation can damage the connective tissue, leading to thickening and hardening of the skin.
- Idiopathic: In some cases, the cause of Scleredema adultorum is unknown. This is referred to as idiopathic Scleredema adultorum.
- Kidney disease: Chronic kidney disease can cause scleredema adultorum. The exact mechanism by which kidney disease causes scleredema adultorum is not yet fully understood.
- Hepatic dysfunction: Hepatic dysfunction can cause scleredema adultorum. The exact mechanism by which hepatic dysfunction causes scleredema adultorum is not yet fully understood.
- Thyroid disorders: Thyroid disorders such as hypothyroidism or hyperthyroidism can cause scleredema adultorum. The exact mechanism by which thyroid disorders cause scleredema adultorum is not yet fully understood.
- Alcohol abuse: Chronic alcohol abuse can lead to scleredema adultorum. It is believed that the chronic inflammation caused by alcohol abuse can lead to hardening of the skin.
- Genetic factors: Genetic factors may play a role in the development of scleredema adultorum. However, the exact genes involved in the
Symptoms
Most common symptoms of scleredema adultorum:
- Thickening and hardening of the skin: One of the most obvious symptoms of scleredema adultorum is thickening and hardening of the skin. This can be most noticeable on the neck, upper arms, and upper back.
- Skin that feels tight: The skin affected by scleredema adultorum can feel tight and stretched, making it difficult to move.
- Skin that appears shiny: The skin may appear shiny or waxy due to the thickening of the skin.
- Reddish-brown or yellowish-brown patches on the skin: These patches may be present on the skin, and they can be a result of the thickening of the skin.
- Difficulty moving the affected areas: Due to the stiffness and tightness of the skin, it can be difficult to move the affected areas.
- Joint pain: Joint pain may be present in patients with scleredema adultorum due to the stiffness of the skin.
- Swelling: Swelling can occur in the affected areas due to the thickening of the skin.
- Limited range of motion: Due to the stiffness of the skin, patients may have a limited range of motion.
- Fatigue: Fatigue may be present in patients with scleredema adultorum, but the reason for this is not well understood.
- Weight loss: Weight loss may be present in some patients with scleredema adultorum, but the reason for this is not well understood.
- Numbness or tingling in the affected areas: Numbness or tingling may be present in the affected areas due to the thickening of the skin.
- Itching: Itching may occur in the affected areas due to the thickening of the skin.
- Skin that is more sensitive to pressure: The skin may be more sensitive to pressure, making it uncomfortable to wear tight clothing or shoes.
- Dry skin: The skin may become dry due to the thickening of the skin.
- Skin that is more prone to infections: The thickening of the skin can make it more prone to infections.
- Changes in skin color: The skin may become lighter or darker in color due to the thickening of the skin.
- Ulcers: Ulcers may develop on the skin due to the thickening of the skin.
- Difficulty swallowing: In rare cases, patients with scleredema adultorum may have difficulty swallowing due to the thickening of the skin in the throat.
- Difficulty breathing: In rare cases, patients with scleredema adultorum may have difficulty breathing due to the thickening of the skin around the chest.
- Vision problems: In rare cases, patients with scleredema adultorum may experience vision problems due to the thickening of the skin around the eyes.
Diagnosis
Potential diagnoses and tests for scleredema adultorum and explain each in detail.
- Clinical examination: Clinical examination is the first step in diagnosing scleredema adultorum. A physical examination of the skin can reveal areas of thickening and hardening, which are characteristic of scleredema adultorum. The affected skin is typically firm to the touch and does not pit with pressure.
- Skin biopsy: A skin biopsy can be performed to confirm the diagnosis of scleredema adultorum. The biopsy will reveal a thickening of the dermis and increased collagen deposition.
- Blood tests: Blood tests can help identify underlying conditions that may be associated with scleredema adultorum, such as diabetes, monoclonal gammopathy, and lymphoproliferative disorders.
- Serum protein electrophoresis: Serum protein electrophoresis is a blood test that can help detect monoclonal gammopathy, a condition in which abnormal proteins are produced by a single clone of cells.
- Complete blood count: A complete blood count can help identify eosinophilia, which is commonly seen in eosinophilic fasciitis, another connective tissue disorder that shares symptoms with scleredema adultorum.
- Imaging tests: Imaging tests such as X-rays, CT scans, and MRIs can be helpful in identifying areas of thickening in the skin, fascia, and underlying tissues.
- Magnetic resonance imaging (MRI): An MRI can help identify the extent of skin thickening and determine if there is involvement of the underlying muscles and bones.
- High-resolution ultrasound: High-resolution ultrasound can be used to visualize the thickness of the skin and underlying tissues.
- Electromyography (EMG): Electromyography can be used to assess muscle function and detect any involvement of the underlying muscles.
- Nerve conduction studies: Nerve conduction studies can be used to evaluate nerve function and detect any involvement of the peripheral nerves.
- Pulmonary function tests: Pulmonary function tests can be helpful in identifying lung involvement, which can occur in rare cases of scleredema adultorum.
- Echocardiography: Echocardiography can be used to evaluate heart function and detect any involvement of the heart muscle.
- Spirometry: Spirometry can be used to evaluate lung function and detect any involvement of the lungs.
- Urinalysis: Urinalysis can help detect any kidney involvement, which can occur in rare cases of scleredema adultorum.
- Electrocardiogram (ECG): An ECG can be used to evaluate heart function and detect any involvement of the heart muscle.
- Skin surface temperature measurement: Skin surface temperature measurement can help identify areas of skin thickening that are warmer than surrounding tissue, which is a characteristic finding in scleredema adultorum.
- Skin hydration measurement: Skin hydration measurement can be used to assess skin elasticity, which is often decreased in scleredema adultorum.
- Laser Doppler flowmetry: Laser Doppler flowmetry can be used to assess blood flow in the skin and detect any abnormalities.
- Skin impedance measurement: Skin impedance measurement can be used to assess the electrical resistance of the skin, which is often increased in scleredema adultorum.
Treatment
Treatments for scleredema adultorum and their details.
- Physical Therapy: Physical therapy involves exercises, stretching, and massage to improve mobility and reduce stiffness in affected joints. It can also help to prevent muscle atrophy and reduce pain.
- Occupational Therapy: Occupational therapy aims to improve the daily functioning of patients by focusing on the activities of daily living. It helps patients to perform basic tasks like dressing, grooming, and feeding.
- Moisturizers: Moisturizers can help to prevent skin dryness and cracking, which is common in scleredema adultorum. Moisturizers containing urea or lactic acid are particularly helpful in improving skin hydration.
- Topical Steroids: Topical steroids are often prescribed to reduce skin inflammation and itching. They are available in various forms such as creams, ointments, and lotions.
- Topical Calcineurin Inhibitors: Topical calcineurin inhibitors are a newer class of medication used to treat inflammatory skin disorders. They work by reducing inflammation and preventing further damage to the skin.
- Oral Steroids: In severe cases, oral steroids may be prescribed to reduce inflammation throughout the body. However, long-term use of oral steroids can cause serious side effects, so their use is limited.
- Immunosuppressive Therapy: Immunosuppressive therapy involves the use of medications that suppress the immune system. This is usually reserved for severe cases of scleredema adultorum that do not respond to other treatments.
- Methotrexate: Methotrexate is an immunosuppressive medication that is sometimes used to treat scleredema adultorum. It works by suppressing the immune system and reducing inflammation.
- Cyclosporine: Cyclosporine is another immunosuppressive medication that may be used to treat scleredema adultorum. It works by suppressing the immune system and reducing inflammation.
- Phototherapy: Phototherapy involves the use of ultraviolet light to treat skin conditions. It is sometimes used to treat scleredema adultorum, as it can help to reduce inflammation and improve skin mobility.
- Extracorporeal Photopheresis: Extracorporeal photopheresis is a type of phototherapy that involves removing blood from the patient, treating it with ultraviolet light, and returning it to the patient. This treatment has been shown to be effective in some cases of scleredema adultorum.
- Physical Modalities: Physical modalities such as heat therapy, cold therapy, and ultrasound can be helpful in reducing pain, stiffness, and inflammation in affected joints.
- Anti-inflammatory Medications: Nonsteroidal anti-inflammatory medications such as ibuprofen and naproxen can help to reduce inflammation and pain in the affected areas.
- Antihistamines: Antihistamines can help to reduce itching and improve sleep quality in patients with scleredema adultorum.
- Antibiotics: Antibiotics may be prescribed to treat bacterial infections that can occur in the affected areas of the skin.
- Wound Care: Wound care is important in patients with scleredema adultorum, as the skin can become prone to cracking and infection. Proper wound care includes cleaning the affected area, applying appropriate dressings, and monitoring for signs of infection.
