Scleroderma

Background:
The Foundation has recently received some feedback from some members of the public asking for more information regarding the condition known as Calcinosis. As part of our efforts we sought to provide a brief, yet informative insight into the topic.

Calcinosis is one of the symptoms of CREST Syndrome, which is a form of Systemic Scleroderma, and is the term for the form of the disease that not only includes the skin, but also involves the tissues beneath, the blood vessels, and the major organs. Systemic Scleroderma is typically broken down into Limited Coetaneous Scleroderma and Diffuse Coetaneous Scleroderma.

Limited coetaneous Scleroderma typically comes on gradually and affects the skin only in certain areas: the fingers, hands, face, lower arms, and legs. Most people with limited disease have Raynaud’s phenomenon for years before skin thickening starts. Telangiectasia and Calcinosis often follow.

What is Calcinosis:
Calcinosis itself, is the formation of calcium deposits (calcium phosphate) in the connective tissues. Where this condition commonly occurs in the skin, it is known as Calcinosis Cutis or Cutaneous Calcification. These aforementioned deposits are typically found on the fingers, hands, face, and trunk and on the skin above elbows and knees.

When the deposits break through the skin, painful ulcers can result. Despite some beliefs, it is not caused by too much calcium in the diet, and a few conditions other than Scleroderma may also cause Calcinosis. These would include for example, dermatomyositis, lupus, Vitamin D, tumors, and parasitic infections.

Calcium phosphate crystals have a remarkable tendency to aggregate into snowball-like clumps and are invariably associated with particular collagens. Collagens are those fibrous, insoluble proteins found in the connective tissues, including skin, bone, ligaments, and cartilage. More than 80% (dry weight) of our skin is made of collagen, and it also forms about 30% of the body's total protein.

What Causes Calcinosis Cutis:
We thought it best to focus on Calcinosis Cutis, and its causes. Calcinosis cutis is divided into four major types according to the original cause of the symptoms.

Dystrophic calcinosis cutis occurs in an area where there is damaged, inflamed, neoplastic or necrotic skin. Tissue damage may be from mechanical, chemical, infectious or other factors. Normal serum calcium and phosphate levels exist. Conditions that can cause calcinosis include:

• Trauma
• Acne
  
• Varicose veins
• Infections
• Tumours (pilomatrixoma, cysts, basal cell carcinomas and others)
• Connective tissue disease (dermatomyositis, systemic sclerosis, cutaneous lupus erythematosus)
• Panniculitis
  
• Inherited diseases of connective tissue (Ehlers-Danlos syndrome, Werner syndrome, Pseudoxanthoma elasticum, Rothmund-Thomson syndrome)
  

Metastatic calcinosis cutis occurs in the setting of abnormal calcium and phosphate metabolism and is often associated with hypercalcaemia and/or hyperphosphataemia. Conditions that can cause calcinosis include:

• Primary or secondary hyperparathyroidism
• Paraneoplastic hypercalcaemia
• Destructive bone disease, e.g. Paget disease
• Milk-alkali syndrome
• Excessive vitamin D intake
• Sarcoidosis
  
• Chronic renal failure
• Calciphylaxis
  

Idiopathic calcinosis cutis generally occurs in the absence of any known tissue injury or systemic metabolic defect. Calcification is usually localised to one general area.

Iatrogenic calcinosis cutis Calcinosis that arises secondary to a treatment or procedure, e.g. parenteral administration of calcium or phosphate, calcium deposition in newborns from repeated heel sticks.

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