Scleredema diabeticorum is a rare skin complication that occurs in people with poorly controlled diabetes. The condition is characterized by thickening and hardening of the skin on the back of the neck, upper back, and shoulders. The skin can become tight and immobile, which can cause discomfort and limited range of motion.
There are different types of scleredema diabeticorum, each with its own set of characteristics and symptoms. In this article, we will discuss the definitions and types of scleredema diabeticorum.
- Scleredema adultorum – Scleredema adultorum is the most common type of scleredema diabeticorum. It usually occurs in people with poorly controlled diabetes, particularly those with longstanding disease. The condition is characterized by thickening and hardening of the skin on the back of the neck, upper back, and shoulders. The skin can feel tight and have a “woody” texture. Scleredema adultorum typically does not cause any other symptoms besides skin changes.
- Scleredema neonatorum – Scleredema neonatorum is a rare type of scleredema diabeticorum that occurs in newborns. The condition is characterized by diffuse thickening and hardening of the skin on the face, neck, and upper back. The skin can feel tight and immobile, which can interfere with feeding and breathing. Scleredema neonatorum is typically associated with poorly controlled maternal diabetes during pregnancy.
- Scleredema diabeticorum of Buschke – Scleredema diabeticorum of Buschke is a rare type of scleredema diabeticorum that usually occurs in men with poorly controlled diabetes. The condition is characterized by diffuse thickening and hardening of the skin on the upper body, including the chest, shoulders, and upper back. The skin can feel tight and immobile, which can cause discomfort and limited range of motion. Scleredema diabeticorum of Buschke is typically associated with poor glycemic control and long-standing diabetes.
- Scleredema diabeticorum of Anders – Scleredema diabeticorum of Anders is a rare type of scleredema diabeticorum that usually occurs in women with poorly controlled diabetes. The condition is characterized by thickening and hardening of the skin on the upper body, including the chest, shoulders, and upper back. The skin can feel tight and immobile, which can cause discomfort and limited range of motion. Scleredema diabeticorum of Anders is typically associated with poor glycemic control and long-standing diabetes.
- Scleredema adultorum of Buschke – Scleredema adultorum of Buschke is a rare type of scleredema diabeticorum that usually occurs in men. The condition is characterized by diffuse thickening and hardening of the skin on the upper body, including the chest, shoulders, and upper back. The skin can feel tight and immobile, which can cause discomfort and limited range of motion. Scleredema adultorum of Buschke is not necessarily associated with diabetes, but it can occur in people with the condition.
- Scleredema adultorum of Anders – Scleredema adultorum of Anders is a rare type of scleredema diabeticorum that usually occurs in women. The condition is characterized by thickening and hardening of the skin on the upper body, including the chest, shoulders, and upper back. The skin can feel tight and immobile, which can cause discomfort and limited range of motion. Scleredema adultorum of Anders is not necessarily associated with diabetes, but it can occur in people with the condition.
Causes
While the exact cause of SD is unknown, it is thought to be associated with diabetes mellitus. Here are potential causes and risk factors for SD, along with an explanation of each:
- Diabetes Mellitus: SD is most commonly seen in individuals with poorly controlled diabetes mellitus, particularly type 2 diabetes.
- Poor Glycemic Control: Chronic hyperglycemia can lead to the accumulation of advanced glycation end products (AGEs) in the skin, which may contribute to the development of SD.
- Insulin Resistance: Insulin resistance, a hallmark of type 2 diabetes, may also play a role in the pathogenesis of SD.
- Obesity: Obesity is a risk factor for both type 2 diabetes and SD, and may contribute to the development of both conditions.
- Age: SD is more common in middle-aged and elderly individuals, which may be related to the increased incidence of type 2 diabetes in these age groups.
- Sex: SD is more common in men than in women, although the reason for this is not well understood.
- Race/Ethnicity: SD has been reported in individuals of all races and ethnicities, but may be more common in certain populations, such as African Americans.
- Autoimmunity: Although SD is not considered an autoimmune disorder, there is some evidence to suggest that autoimmunity may play a role in its development.
- Inflammation: Inflammation is thought to be involved in the pathogenesis of SD, and may be related to the presence of chronic hyperglycemia and insulin resistance.
- Infection: SD has been reported in association with a number of infections, including streptococcal infections, viral infections, and mycoplasma infections.
- Medications: There have been reports of SD occurring in association with certain medications, including statins, antipsychotics, and chemotherapy drugs.
- Trauma: Trauma to the skin, such as from surgery or radiation therapy, may trigger the development of SD.
- Genetic Predisposition: There may be a genetic predisposition to the development of SD, although no specific genes have been identified.
- Environmental Factors: Exposure to certain environmental factors, such as chemicals or toxins, may increase the risk of developing SD.
- Hormonal Imbalances: Hormonal imbalances, such as those seen in thyroid disorders, may contribute to the development of SD.
- Metabolic Disorders: Other metabolic disorders, such as hyperlipidemia or metabolic syndrome, may increase the risk of developing SD.
- Neuropathy: Neuropathy, a common complication of diabetes, may be related to the development of SD.
- Vascular Abnormalities: Vascular abnormalities, such as those seen in atherosclerosis or peripheral vascular disease, may contribute to the development of SD.
- Immune System Dysfunction: Dysfunction of the immune system, which is commonly seen in individuals with diabetes, may be related to the development of SD.
- Stress: Chronic stress has been associated with the development of a number of skin disorders, and may contribute to the development of SD.
Symptoms
Symptoms of scleredema diabeticorum, along with a detailed explanation of each symptom.
- Hardening and thickening of the skin: The hallmark symptom of scleredema diabeticorum is the hardening and thickening of the skin. This usually occurs on the back of the neck, upper back, and shoulders.
- Restricted mobility: As the skin becomes thicker and harder, it may become more difficult to move the affected areas. This can result in restricted mobility and difficulty with daily activities.
- Discoloration of the skin: The skin may take on a reddish or brownish hue, especially in the early stages of the condition.
- Itching: The thickened skin may feel itchy, which can be bothersome and lead to scratching that can further damage the skin.
- Pain or discomfort: In some cases, the thickened skin can cause pain or discomfort.
- Fatigue: Some people with scleredema diabeticorum may experience fatigue or a general feeling of malaise.
- Fever: In rare cases, a fever may develop.
- Numbness or tingling: Scleredema diabeticorum can sometimes affect the nerves, leading to numbness or tingling in the affected areas.
- Swelling: The thickened skin may cause swelling in the affected areas.
- Dry skin: The skin may become dry and rough to the touch.
- Skin cracking: In severe cases, the thickened skin may crack or split, leading to further discomfort and the risk of infection.
- Ulcers: In rare cases, scleredema diabeticorum can cause ulcers to form on the skin.
- Skin infections: The thickened skin may be more susceptible to infection, which can further complicate the condition.
- Limited range of motion: As the skin becomes thicker and less flexible, it may become more difficult to move the affected areas, resulting in a limited range of motion.
- Muscle weakness: In some cases, the thickened skin can cause muscle weakness in the affected areas.
- Difficulty breathing: If the condition affects the neck, it may cause difficulty breathing or swallowing.
- Vision problems: Rarely, scleredema diabeticorum can affect the eyes, leading to vision problems.
- Heart problems: In very rare cases, scleredema diabeticorum can affect the heart, leading to problems such as heart failure or arrhythmia.
- Joint pain: The thickened skin may cause joint pain in the affected areas.
- Weight loss: In rare cases, scleredema diabeticorum may be accompanied by weight loss, which can be a sign of more serious complications.
Diagnosis
Different diagnostic tests and approaches to help identify scleredema diabeticorum.
- Clinical examination The first step in diagnosing scleredema diabeticorum is a thorough clinical examination by a qualified healthcare professional. During this examination, the healthcare professional will evaluate the skin in the affected areas and look for any signs of hardening, thickening, or discoloration. They may also ask the patient about their medical history and any symptoms they are experiencing.
- Skin biopsy A skin biopsy involves removing a small sample of skin from the affected area for laboratory analysis. This test can help confirm the diagnosis of scleredema diabeticorum and rule out other conditions that may have similar symptoms. The biopsy may show thickened collagen fibers in the skin, which is a hallmark of this condition.
- Blood tests Blood tests can help identify diabetes mellitus and monitor blood glucose levels. Elevated levels of hemoglobin A1C and fasting blood glucose levels are often seen in patients with scleredema diabeticorum.
- Imaging studies Imaging studies such as X-rays, CT scans, and MRI scans can help identify any underlying complications associated with diabetes mellitus. For example, these tests may reveal diabetic neuropathy, which can contribute to the development of scleredema diabeticorum.
- Glycosylated hemoglobin (HbA1c) test This test measures the amount of hemoglobin in the blood that has glucose attached to it. It is often used to monitor blood glucose levels in individuals with diabetes mellitus.
- Oral glucose tolerance test (OGTT) An OGTT measures the body’s ability to metabolize glucose. This test involves drinking a sugary drink and then having blood drawn at intervals over several hours. Elevated blood glucose levels may be a sign of diabetes mellitus.
- Blood lipid profile A blood lipid profile measures the levels of cholesterol and triglycerides in the blood. High levels of these substances are often seen in individuals with diabetes mellitus and can contribute to the development of scleredema diabeticorum.
- Renal function tests Diabetes mellitus can cause damage to the kidneys. Renal function tests such as creatinine and blood urea nitrogen (BUN) can help identify any kidney damage and monitor kidney function.
- Electrocardiogram (ECG) An ECG measures the electrical activity of the heart. Diabetes mellitus can increase the risk of heart disease, so an ECG may be ordered to monitor heart function.
- Chest X-ray A chest X-ray can help identify any abnormalities in the lungs or chest that may be contributing to the development of scleredema diabeticorum.
- Spirometry Spirometry is a test that measures lung function. Individuals with diabetes mellitus may be at increased risk of respiratory complications, so spirometry may be ordered to assess lung function.
- Nerve conduction studies Nerve conduction studies can help identify any damage to the nerves that may be contributing to the development of scleredema diabeticorum. This test measures the speed at which nerves conduct electrical signals.
- Electroencephalogram (EEG) An EEG measures the electrical activity of the brain. Diabetes mellitus can increase the risk of neurological complications, so an EEG may be ordered to monitor brain function.
- Retinal exam Diabetes mellitus can cause damage to the blood vessels in the eyes, leading to diabetic retinopathy.
Treatment
treatments for scleredema diabeticorum, their mechanisms of action, and their effectiveness.
- Glycemic control: The most important factor in the treatment of scleredema diabeticorum is glycemic control. Tight control of blood glucose levels can prevent the development and progression of scleredema diabeticorum.
- Topical corticosteroids: Topical corticosteroids are often used in the treatment of scleredema diabeticorum, and they have been reported to be effective in reducing skin thickness and hardness. They are thought to act by suppressing inflammation and reducing the accumulation of glycosaminoglycans in the skin.
- Topical calcipotriol: Topical calcipotriol, a vitamin D analog, has been reported to be effective in reducing skin thickness and hardness in patients with scleredema diabeticorum. It is thought to act by inhibiting the proliferation of fibroblasts and reducing the production of extracellular matrix proteins.
- Topical tacrolimus: Topical tacrolimus, an immunosuppressive agent, has been reported to be effective in reducing skin thickness and hardness in patients with scleredema diabeticorum. It is thought to act by reducing the production of extracellular matrix proteins.
- Topical urea: Topical urea has been reported to be effective in reducing skin thickness and hardness in patients with scleredema diabeticorum. It is thought to act by hydrating the skin and reducing the accumulation of glycosaminoglycans.
- Topical dimethyl sulfoxide (DMSO): Topical DMSO has been reported to be effective in reducing skin thickness and hardness in patients with scleredema diabeticorum. It is thought to act by reducing inflammation and increasing the penetration of other topical agents.
- Systemic corticosteroids: Systemic corticosteroids have been used in the treatment of scleredema diabeticorum, but their effectiveness is uncertain. They are thought to act by suppressing inflammation and reducing the accumulation of glycosaminoglycans in the skin.
- Systemic immunosuppressive agents: Systemic immunosuppressive agents, such as methotrexate and azathioprine, have been used in the treatment of scleredema diabeticorum, but their effectiveness is uncertain. They are thought to act by reducing the production of extracellular matrix proteins.
- Systemic antibiotics: Systemic antibiotics, such as tetracycline and erythromycin, have been used in the treatment of scleredema diabeticorum, but their effectiveness is uncertain. They are thought to act by reducing inflammation and infection.
- Extracorporeal photopheresis (ECP): ECP is a form of immunomodulatory therapy that involves the removal of white blood cells from the patient’s blood, exposure to ultraviolet A light, and reinfusion into the patient’s bloodstream. ECP has been reported to be effective in reducing skin thickness and hardness in patients with scleredema diabeticorum. It is thought to act by inducing apoptosis of activated T-cells and reducing the production of extracellular matrix proteins.
- Phototherapy: Phototherapy involves exposing the skin to UV light to reduce inflammation and promote healing. It is often used in combination with other treatments for scleredema diabeticorum. Phototherapy is generally safe and effective but may cause sunburn and skin cancer with prolonged exposure.
- Extracorporeal photopheresis: Extracorporeal photopheresis is a type of phototherapy that involves removing blood from the patient, treating it with a light-sensitive drug, and exposing it to UV light before returning it to the patient’s bloodstream. This treatment is used in severe cases of scleredema diabeticorum and may be associated with side effects such as fever and chills.
- Immunomodulatory agents: Immunomodulatory agents are drugs that modify the immune response to reduce inflammation. They may be used in severe cases of scleredema diabeticorum, but their effectiveness in treating the condition is unclear.
- Anti-tumor necrosis factor (TNF) therapy: Anti-TNF therapy is a type of immunomodulatory agent that targets a protein called tumor necrosis factor. It is used to treat autoimmune disorders and may be effective in treating scleredema diabeticorum in some cases.
- Mycophenolate mofetil: Mycophenolate mofetil is an immunosuppressant drug that is used to treat several autoimmune disorders. It may be effective in treating scleredema diabeticorum, but more research is needed to determine its effectiveness.
- Cyclosporine: Cyclosporine is an immunosuppressant drug that is used to prevent organ rejection in transplant patients. It may be effective in treating scleredema diabeticorum, but its use is associated with several side effects, including hypertension, kidney damage, and increased risk of infection.
- Methotrexate: Methotrexate is an immunosuppressant drug that is used to treat several autoimmune disorders. It may be effective in treating scleredema diabeticorum, but its use is associated with several side effects, including liver damage and increased risk of infection.
- Azathioprine: Azathioprine is an immunosuppressant drug that is used to treat several autoimmune disorders. It may be effective in treating scleredema diabeticorum