In calcinosis cutis, calcium phosphate is deposited 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 a condition called calcinosis, calcium salts accumulate in the skin or organs in a pathological manner and become permanently lodged. Calcium is found in the human body primarily as a phophate salt in the form of hydroxyapatite within bone, where it provides strength to bone tissue and serves a reservoir function, meaning it can be released as needed. The deposition of calcium salts in individual tissues and organs may correspond to the accumulation of calcium carbonate and, above all, to the deposition of calcium phosphate. Calcinosis cutis is a calcinosis and manifests itself in the form of calcium phosphate deposition within the skin. Usually, the skin of the extremities is predominantly affected. The pathological phenomenon is the symptom of a superordinate disease and is also called cutaneous calcinosis. Different forms of calcinosis cutis exist:
- Calcinosis metastatica
- Calcinosis metabolica in the form of calcinosis circumscripta or universalis,
- Calcinosis dystrophica
- The idiopathic calcinosis
- The latrogenic calcinosis.
The division into the above subgroups corresponds to a cause-dependent subdivision. Calcinosis cutis is not always used exclusively for skin deposits. The use of the word in clinical practice is multifaceted and at times somewhat diffuse.
Causes
The deposition of calcium phosphate in the skin can have several causes. One conceivable cause is 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, deposition may be the result of infections or occur in the setting of chronic hypercalcemia and hyperphosphatemia due to metabolic disturbances. Calcinosis metastatica is caused by a disorder of calcium phosphate metabolism. Metabolic disorders also characterize calcinosis metabolica. In calcinosis dystrophica, there are no detectable disturbances of calcium metabolism. In this form, calcinosis accompanies tumors, phleboliths, varices, tuberculous lymphomas, or local trauma. Idiopathic calcinoses have no obvious cause and consequently cannot be attributed to any external, or internal, influence. In the case of iatrogenic calcinosis of the skin, the cause is again to be sought in treatment received by the patient for other ailments. A benign calcinosis of unclear cause also exists with 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 is rheumatic processes or ochronosis.
Symptoms, complaints, and signs
Symptoms of calcinosis cutis may vary in severity. In this context, metabolic calcinosis is divided, for example, into the subgroups calcinosis circumscripta and universalis. In the former phenomenon, calcium salts deposit locally in the form of individual nodules in the skin and possibly the joints, where they can cause restricted movement, 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 subcutis may be affected by the deposits. The same applies to organs. This severe form of calcinosis is called lipocalcinogranulomatosis and is not calcinosis cutis in the strict sense. Instead, calcinosis cutis is one of many symptoms of this phenomenon. In the case of calcinosis metastatica, deposits in the internal organs are also conceivable and then usually affect the kidney, stomach or lungs. Due to the deposits, the organs involved may be affected by functional impairment in severe calcinosis. As a rule, the clinical picture of calcinosis cutis is characterized by macroscopically visible, hard palpable swellings.Local inflammation often occurs in addition. Furthermore, epidermal plaque breakthroughs may occur. With calcinosis intervertebralis, calcifications in the intervertebral discs are also conceivable.
Diagnosis
The diagnosis of calcinosis cutis is made on the basis of the clinical presentation, history, and imaging. To confirm the initial tentative diagnosis, a tissue sample may be useful. Laboratory examination of this sample will unmask calcium phosphate. Detection of the primary cause plays a key role in diagnosis. The prognosis of patients depends on the causative disease. The same is true for therapy.
Complications
The course of calcinosis cutis depends largely on its cause and specific localization. The deposition of calcium phosphates in the skin has many different underlying diseases, among which the one that applies to the affected person must be identified. In connection with these – sometimes serious – underlying diseases, further complications may of course occur which go beyond calcinosis cutis. For example, calcium metabolism may be disturbed, but tumors or tuberculosis may also be causative. Among other things, it depends on where calcinosis cutis occurs and how far it spreads. In nodular form it causes complications such as pain, stiffness and restrictions in mobility. Severe cases also affect the subcutaneous tissue or organs. If the organs are affected, there is no classic calcinosis cutis, since this is limited to the skin in the strict sense. However, they often interact with each other, and calcinosis cutis may be a sign of more severe calcinosis. This can cause dysfunction of the kidney, stomach or lungs. These organs are affected in most cases. While calcinosis in the skin can be removed without complications comparatively, calcinosis of the organs requires more complicated surgical procedures.
When should you see a doctor?
As a rule, self-healing does not occur in calcinosis cutis. For this reason, medical treatment is definitely necessary for this disease to avoid further complications and discomfort. The doctor should be consulted when there is the formation of nodules on the skin. Also the joints can be affected, so that it possibly comes to restrictions in the movement with the patient. A doctor should also be consulted if the joints are very painful or if the patient suffers from permanent stiffness. Most patients also suffer from inflammation or swelling. If these occur without any particular reason, an examination by a physician 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 symptoms. In some cases, calcinosis cutis can also have a negative effect on the internal organs, so that these must also be treated.
Treatment and therapy
As a rule, calcinosis cutis is treated surgically. Accordingly, invasive removal of the deposits is the focus of therapy. When calcinosis is confined to the skin, this procedure is more or less simple and usually does not require hospital admission. Often, treatment can be performed under local anesthesia of the affected skin areas. In the case of organ involvement, removal is more difficult. In addition to general anesthesia, this requires precise planning of the procedure, which usually takes place with the aid of imaging procedures such as MRI. The removal of calcium deposits is only a symptomatic treatment. Therefore, the procedure must be accompanied by causative therapy of the primary disease. Acute hypercalcemia can often be controlled by the administration of loop diuretics and glucocorticoids, as this supports renal excretion. For some causes of calcinosis, no causal therapies are available, so treatment in these cases is purely symptomatic. The same is true for idiopathic calcinosis whose cause is not discovered.
Outlook and prognosis
The causative disease as well as the extent of calcium involvement are essential in establishing a prognostic outlook for calcinosis cutis. Many patients do not achieve permanent freedom from symptoms because chronic disease or systemic disorders are diagnosed.Here, a chronic continuous prognosis is given. In many of these patients, the calcium level in the organism is treated and monitored in a long-term therapy. Relief of symptoms occurs and surgical interventions can be avoided or reduced. If the underlying disease is progressive, the symptoms recur despite removal of the calcium. Surgical removal of the plaque is performed repeatedly throughout life. A metabolic disorder can be treated well with medication. Nevertheless, recurrence is also very likely in this case. If calcinosis cutis has developed as a result of infection following skin injury, the best prognosis prospects exist. In most cases, the patient experiences permanent relief of symptoms after surgery and removal of the plaque. If no sequelae are present, the patient is discharged from treatment as cured within a few weeks. The prognosis worsens if tumors or tuberculosis are causally responsible for calcinosis cutis. Despite numerous medical advances, there is a potential risk to the life of the affected individual.
Prevention
Calcionsis cutis cannot be universally prevented because not all causes of the phenomenon have been detected to date.
Aftercare
Follow-up care is possible in some cases of calcinosis cutis. In any case, the affected person should avoid the occurrence of the disease and find out the cause of the deposits in order to stop them completely. Surgical treatment of calcinosis cutis usually occurs without complications and does not require hospitalization, so that follow-up care is no longer necessary in this case. However, even after treatment, regular visits to the doctor are necessary to avoid further complications. The disease can also be treated by taking medication, although the patient must ensure that he or she takes the medication regularly in order to permanently alleviate the symptoms. In some cases, a causal therapy is not possible, so that only symptomatic treatment can be given. The life expectancy of the patient is usually not negatively affected by the disease. Furthermore, calcinosis cutis can also lead to psychological complaints or depression. Especially in children, bullying or teasing often occurs, so that psychological treatment may also be useful. Parents can also participate in psychological treatment in the case of calcinosis cutis and support the child.
This is what you can do 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 surgery. 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. Accompanying this, the diet must be changed. Which diet is suitable in detail depends on the severity of the skin calcifications. In the case of mild symptoms, it is sufficient to avoid foods and drinks that irritate the skin. Thus, no overly salty or strongly spiced foods should be consumed. Alcohol and caffeine are also best avoided. In consultation with the dermatologist, various natural remedies can be used. Aloe vera and preparations with devil’s claw as well as compresses with chamomile or sage have proven themselves. Measures such as yoga or qigong stimulate the blood flow and thus support the calcinosis cutis therapy. If the measures described do not have the desired effect or if calcifications of the skin occur again, the dermatologist must be consulted. Patients should consult the physician closely and involve other specialists so that the necessary treatment measures can be initiated immediately in the event of any accompanying symptoms.
