What Is Scleredema?
Scleredema is a form of cutaneous mucinosis having an unknown cause. Cutaneous mucinosis includes a diverse group of skin conditions occurring due to the accumulation of mucin, a jelly-like complex carbohydrate substance present on the skin. The common signs and symptoms shown are hardening and tightening of the skin and restricting movements. In addition, the skin on the affected region appears red or brown with an orange skin appearance.
What Are the Types of Scleredema?
There are three types of scleredema. All three types of scleredema can restrict movement, but serious consequences are rarely shown in some cases. Occasionally, swallowing and speech are affected. Other organs such as the eyes, tongue, parotid gland, muscles, joints, and heart can also be affected.
Type 1 Scleredema:
Type one is an acute type of scleredema. It originates after a Streptococcus pyogenes infection. Hardening on the skin of the face and neck develops quickly and spreads to the upper trunk and arms. This condition spontaneously resolves within six months to two years and commonly affects middle-aged women and children.
Type 2 Scleredema:
Type 2 scleredema does not remain associated with infection. Instead, it develops more slowly and persists without showing any predominant symptoms in the body.
Type 3 Scleredema:
Type 3 scleredema, otherwise known as scleredema diabeticorum, often develops in diabetic patients. The neck, shoulder, arms, and upper back skin gets thick slowly over months or years. The alternative names of scleredema diabeticorum are scleredema of buschke, scleredema adultorum, and diabetic scleredema.
What Is the Difference Between Scleredema and Scleroderma?
Scleroderma refers to a group of rare diseases that cause abnormal connective tissue growth, resulting in patches of tight skin. Scleroderma also results from overproduction and accumulation of collagen in the body tissues. In most cases, scleroderma occurs due to genetics, environmental triggers, and immune system problems.
There are different types of scleroderma, which often affect females. Signs and symptoms of scleroderma vary according to the type of scleroderma. In some cases, scleroderma affects only the skin. In other cases, it can also cause damage to the internal structures such as blood vessels and other internal organs. Doctors diagnose scleroderma by the patient’s medical history, physical examination, laboratory tests, and skin biopsy. Though there is no cure, various treatments help to control symptoms and complications.
Scleredema presents as ill-defined, woody, and non-pitting plaques on the skin. The affected skin appears red or brown with an orange skin appearance. The face may appear expressionless with difficulty in opening the mouth. The rapidity of onset and locations of involvement differ based on the type of scleredema. It is often diagnosed by skin biopsy, and treatment varies according to the type of scleredema.
Though scleredema and scleroderma look similar, they differ in their histologic appearance. The scleredema is differentiated from scleroderma by the presence of mucin without skin adnexa destruction.
What Are the Causes of Scleredema Diabeticorum?
The exact cause behind scleredema diabeticorum is unknown. But this condition often tends to occur more commonly in males having uncontrolled diabetes, obesity, people using insulin, and other people facing diabetic complications.
Other types of scleredema can develop in people experiencing,
1) Febrile illness with streptococcus infection.
2) Trauma.
3) Multiple myeloma.
4) Drug reaction.
Scleredema also develops in people having other systemic diseases such as,
2) Hyperparathyroidism.
3) Sjogren syndrome.
4) Rheumatoid arthritis.
5) Malignant insulinoma.
6) HIV (human immunodeficiency virus) infection.
What Are the Symptoms of Scleredema Diabeticorum?
The symptoms develop slowly. Over time, the affected person may notice,
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Thick, hard skin that remains smooth.
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Reddish painless lesions occur on the same site on both sides of the body.
In severe cases, thickened skin can cause difficulty moving the upper body with breathing problems. Some people may face problems in doing a clenched fist because the skin on the back of the hand may remain tight.
How to Diagnose Scleredema Diabeticorum?
A person may consult a doctor when he faces difficulty controlling blood sugar and notices any symptoms of scleredema. The doctor might do a physical examination and ask about medical history and other symptoms faced by the patient.
No imaging tests are required for diagnosing scleredema. The other diagnostic tests advised are fasting blood sugar, glucose intolerance, A1C (glycated hemoglobin), and skin biopsy. Mucin deposits between collagen bundles in the dermis are shown in skin biopsy. In some cases, imaging tests are required when scleredema occurs associated with internal malignancies.
How Is Scleredema Diabeticorum Treated?
There is no specific treatment for this condition. The treatments that may help are,
1) Consistent control of blood sugar.
2) Phototherapy, a procedure where the skin is exposed carefully to ultraviolet light.
3) Taking Glucocorticoid medications, either orally or topically.
4) Electron beam therapy (a type of radiation therapy).
5) Medicines that suppress the immune system.
6) Physical therapy is when a person faces trouble moving their torso or breathing deeply.
What Are the Complications Faced Due to Scleredema?
The complications faced are a limited range of motion, poor wound healing, recurrent skin infections, dysarthria, dysphagia, difficulty in closing the eyes, and restrictive lung disease.
What Is the Prognosis of This Condition?
This condition is incurable, and treatment may improve breathing and movement.
Though scleredema is a benign condition limited to the skin, it can cause limitations in movement. Reduced joint mobility, difficulty opening the mouth and eyes, or restrictive lung disease occurs in extensive trunk involvement. Scleredema on the tongue can cause dysarthria, difficulty in masticating, and tongue protrusion. The pharynx, esophagus, skeletal and cardiac muscle may also be affected.
Scleredema can cause:
1) Cardiomyopathy.
2) Heart failure.
3) Arrhythmias.
4) Pericardial effusion.
5) Unexplained murmurs in the cardiac muscle.
How to Prevent the Development of Scleredema Diabeticorum?
Keeping blood sugar levels within range prevents the development of diabetic complications. However, scleredema may also occur when blood sugar levels remain well controlled. The doctor can provide medications that allow insulin to work better in the body by reducing insulin doses.
Conclusion:
Scleredema diabeticorum is a skin problem commonly occurring in people affected with diabetes. In uncontrolled diabetes, scleredema diabeticorum causes thickened skin with difficulty moving the body and breathing. So, diabetic patients have to take their medications appropriately, and keeping their blood sugar under control through exercise and diet reduces the risk of developing scleredema diabeticorum.
