In calcinosis cutis, calcium phosphate builds up in the skin. The causes are complex and include, for example, disorders of calcium metabolism. Treatment consists of surgical removal of the deposits and therapy for their primary cause.

What is calcinosis cutis?

In so-called calcinosis, calcium salts accumulate in a pathological way in the skin or in the organs and become permanent. Calcium occurs in the human body mainly as phosphate salt in the sense of hydroxyapatite within the bone, where it ensures the strength of bone tissue and fulfills a reservoir function, i.e. can be released when required.

The deposition of calcium salts in individual tissues and organs can, in addition to the accumulation of calcium carbonate, primarily correspond to the deposition of calcium phosphate. Calcinosis cutis is a calcinosis and manifests itself in the form of calcium phosphate deposits within the skin. The skin of the extremities is mostly affected.

The pathological phenomenon is the symptom of a higher-level disease and is also known as skin calcinosis. There are different forms of calcinosis cutis:

• Calcinosis metastatica

• Calcinosis metabolica in the form of a calcinosis circumscripta or universalis,

• Calcinosis dystrophica

• Idiopathic calcinosis

• The latrogenic calcinosis.

The subdivision into the named subgroups corresponds to a cause-dependent breakdown. The calcinosis cutis is not always used exclusively for skin deposits. The use of the word in everyday clinical practice is complex and sometimes somewhat diffuse.

Causes

Calcium phosphate build-up in the skin can have a number of causes. A possible cause are systemic diseases such as dermatomyositis, scleroderma syndrome in the form of CREST syndrome or progressive systemic scleroderma. In addition, the substance can accumulate after previous skin injuries.

In addition, the deposits can be the result of infections or occur in the context of chronic hypercalcemia and hyperphosphataemia due to metabolic disorders. Calcinosis metastatica is caused by a disturbance of the calcium phosphate metabolism. Metabolic disorders also shape the calcinosis metabolica.

In calcinosis dystrophica there are no detectable disorders of calcium metabolism. In this form, calcinosis accompanies tumors, phlebolites, varices, tuberculous lymphomas or local trauma. Idiopathic calcinoses have no obvious cause and therefore cannot be traced back to an external or internal influence.

In the case of iatrogenic calcinosis of the skin, the cause is again to be sought in a treatment that the patient has received for other complaints. A benign calcinosis of unknown cause is also the tumorous calcinosis of Teutschländer’s disease, which causes slow tumor growth in soft tissues with adjacent large joints and is due to mutations in the coding genes for GALNT3, FGF23 and KLOTHO. Another cause are rheumatic processes or ochronosis.

Symptoms, ailments & signs

According to DigoPaul, the symptoms of calcinosis cutis can vary in severity. In this context, metabolic calcinoses are divided into the subgroups Calcinosis circumscripta and universalis, for example. In the case of the first phenomenon, the calcium salts are locally limited in the form of individual nodules in the skin and possibly the joints, where they can cause restricted mobility, pain or stiffness.

This form of calcinosis is also known as calcinosis localisata. Calcinosis universalis, generalisata or diffusa is associated with numerous deposits in the skin. In addition, the subcutaneous tissue can be affected by the deposits. The same goes for organs. This severe form of calcinosis is known as lipocalcinogranulomatosis and, in the narrower sense, is not calcinosis cutis.

On the other hand, the calcinosis cutis is one of the many symptoms of this phenomenon. Deposits in the internal organs are also conceivable in calcinosis metastatica and then usually affect the kidneys, stomach or lungs. The organs involved can be affected by functional impairments in severe calcinosis.

As a rule, the clinical picture of calcinosis cutis is characterized by macroscopically visible, hard palpable swellings. Often there is also local inflammation. Epidermal plaque breakthroughs can also occur. With calcinosis intervertebralis, calcium deposits in the intervertebral discs are also conceivable.

Diagnosis

The diagnosis of calcinosis cutis is based on the clinical picture, medical history, and imaging. A tissue sample can be helpful in confirming the first suspected diagnosis. Laboratory testing of this sample reveals the calcium phosphate. Detection of the primary cause plays a key role in diagnostics. The patient’s prognosis depends on the underlying disease. The same goes for therapy.

Complications

The course of a calcinosis cutis depends largely on its cause and the specific location. The deposition of calcium phosphates in the skin has many different underlying diseases, among which the one that applies to the individual must be identified. In connection with these – sometimes serious – underlying diseases, further complications can of course occur that go beyond the calcinosis cutis.

The calcium metabolism can be disturbed, for example, but tumors or tuberculosis can also be the cause. This depends, among other things, on where the calcinosis cutis occurs and how far it spreads. In the form of nodules, it causes complications such as pain, stiffness and restrictions in the ability to move. Severe cases also affect the subcutaneous tissue or organs.

If the organs are affected, there is no classic calcinosis cutis, as this is limited to the skin in the narrower sense. However, they often interact with one another and calsinosis cutis can be a sign of more severe calcinosis. This can cause kidney, stomach or lung dysfunction. In most cases, these organs are affected. While calcinoses in the skin can be removed with comparatively no complications, calcinoses of the organs require more complicated surgical interventions.

When should you go to the doctor?

As a rule, calcinosis cutis does not heal itself. For this reason, medical treatment is definitely necessary for this disease in order to avoid further complications and complaints. The doctor should then be seen if lumps develop on the skin. The joints can also be affected, so that there may be restrictions in movement for the patient. A doctor must also be consulted if the joints are severely painful or if the patient is permanently stable.

Most people also have inflammation or swelling. If these occur for no particular reason, an examination by a doctor is also necessary. The diagnosis of the disease can usually be made by a general practitioner. Further treatment depends on the cause and also on the exact complaints. In some cases, the calcinosis cutis can also have a negative effect on the internal organs, so that these must also be treated.

Treatment & Therapy

Usually the calcinosis cutis is treated surgically. The invasive removal of the deposits is accordingly the focus of the therapy. If the calcinosis is restricted to the skin, this procedure is more or less simple and usually does not require hospitalization. The treatment can often be carried out under local anesthesia to the affected areas of the skin.

Removal is more difficult with organ involvement. In addition to general anesthesia, precise planning of the procedure is necessary, which usually takes place with the aid of imaging methods such as MRI. Removal of calcium deposits is only symptomatic treatment. Therefore, the procedure must be accompanied by causal therapy for the primary disease.

Acute hypercalcaemia can often be combated by the administration of loop diuretics and glucocorticoids, as this supports excretion via the kidneys. For some causes of calcinosis, no causal therapies are available, so that treatment in these cases is purely symptomatic. The same is true of idiopathic calcinosis, the cause of which is not discovered.

Outlook & forecast

The causal disease and the extent of the calcium attack are essential for establishing a prognosis for calcinosis cutis. Many patients do not achieve permanent freedom from symptoms because chronic diseases or systemic disorders are diagnosed. A chronically continuous prognosis is given here. In many of these patients, the calcium content in the organism is treated and monitored in long-term therapy. The symptoms are alleviated and surgical interventions can thus be avoided or reduced.

If the underlying disease is progressive, the symptoms recur despite the removal of the calcium. Surgical removal of the plaque is carried out repeatedly over a lifetime. A metabolic disorder can be treated well with medication. However, a recurrence is very likely here too.

If the calcinosis cutis developed as a result of an infection after a skin injury, the best prognosis is possible. In most cases, after surgery and removal of the plaque, the patient will experience permanent relief from symptoms. If there are no sequelae, the patient is discharged from treatment as cured within a few weeks.

The prognosis worsens if tumors or tuberculosis are causally responsible for the calcinosis cutis. Despite numerous medical advances, there is a potential danger to life for those affected.

Prevention

The calcionsis cutis cannot be completely prevented, as not all causes of the phenomenon have been detected so far.

Aftercare

Follow-up care is possible in some cases of calcinosis cutis. The sufferer should avoid the occurrence of the disease in any case and find out the cause of the deposits in order to stop them completely. Surgical treatment of the calcinosis cutis usually takes place without complications and does not require an inpatient stay in the hospital, so that follow-up care is no longer necessary.

However, regular visits to the doctor are necessary even after treatment in order to avoid further complications. The disease can also be fought by taking medication, whereby the person affected must ensure that they are taken regularly in order to permanently alleviate the symptoms.

In some cases, causal therapy is not possible, so only symptomatic treatment can be given. The life expectancy of the patient is usually not negatively influenced by the disease. Furthermore, the calcinosis cutis can also lead to psychological complaints or depression.

Bullying or teasing often occurs, especially with children, so psychological treatment can also be useful. In the case of calcinosis cutis, the parents can also participate in the psychological treatment and support the child in this.

You can do that yourself

Calcinosis cutis is usually treated surgically. The most important self-help measure is to follow the doctor’s instructions regarding personal hygiene and diet after the operation. First of all, the body and especially the skin should not be stressed too much so that no further skin changes and other complications occur.

At the same time, the diet must be changed. Which diet is suitable in detail depends on the severity of the skin calcifications. In the case of mild complaints, it is sufficient to avoid food and drinks that irritate the skin. For example, foods that are too salty or spicy should not be consumed. It is also best to avoid alcohol and caffeine.

In consultation with the dermatologist, various naturopathic remedies can be used. Aloe vera and preparations with devil’s claw as well as toppings with chamomile or sage have proven effective. Measures such as yoga or qigong stimulate the blood flow and thereby support the calcinosis cutis therapy.

If the measures described do not have the desired effect or if skin calcifications occur again, the dermatologist must be consulted. The patients should consult closely with the doctor and consult other specialists so that the necessary treatment measures can be initiated immediately in the event of any accompanying symptoms.