Scleredema

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Scleredema

Scleredema (pronounced skler-uh-dee-muh) is a rare, self-limiting skin condition characterized by hardening of the skin.

Etymology

The term "scleredema" is derived from the Greek words "skleros" meaning hard, and "edema" meaning swelling.

Definition

Scleredema is a connective tissue disorder that involves the accumulation of glycosaminoglycans in the skin, leading to thickening and hardening of the skin. It is often associated with diabetes mellitus, infections, and monoclonal gammopathy.

Symptoms

The main symptom of scleredema is the hardening of the skin, which can affect the neck, face, and upper body. Other symptoms may include difficulty moving the affected areas, and in severe cases, difficulty breathing due to involvement of the chest wall.

Diagnosis

Diagnosis of scleredema is typically based on the clinical presentation and a skin biopsy, which shows increased amounts of collagen and mucin in the dermis.

Treatment

Treatment for scleredema is primarily supportive and may include physical therapy to improve mobility. In some cases, medications such as corticosteroids or immunosuppressants may be used.

Related Terms

  • Dermatology: The branch of medicine dealing with the skin and its diseases.
  • Connective tissue disease: A group of diseases that affect the parts of the body that connect the structures of the body together.
  • Glycosaminoglycans: Large molecules that play a key role in skin hydration and wound repair.
  • Diabetes mellitus: A group of diseases that result in too much sugar in the blood.
  • Infections: The invasion of an organism's body tissues by disease-causing agents.
  • Monoclonal gammopathy: A condition in which an abnormal amount of protein is found in the blood.
  • Collagen: The main structural protein in the extracellular space in the various connective tissues in the body.
  • Mucin: A family of high molecular weight, heavily glycosylated proteins.
  • Corticosteroids: A class of steroid hormones that are produced in the adrenal cortex of vertebrates.
  • Immunosuppressants: Drugs that inhibit or prevent activity of the immune system.

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