Calcinosis cutis is a chronic condition in which deposits of solid calcium salts occur in the tissues of the skin, sometimes with the development of inflammation and limitation of joint mobility. The symptoms of this disease are nodules on various parts of the body, mainly on the upper extremities, of a firm and dense consistency, sometimes painful. Diagnosis is carried out by examining the patient’s skin and conducting X-ray examinations. Treatment depends on the cause of the development of this condition, mainly means are used to normalize mineral metabolism, with significant foci, electrocoagulation and surgical intervention are resorted to.
General information
Calcinosis cutis is a dermatological disease of various etiologies, in which conditions are created in the tissues of the skin for the deposition of calcium salts in solid form. For the first time such a condition was professionally described by the French doctor J. Profishe, therefore, in some sources, the syndrome in which calcifications are deposited in the skin is named after him. This is a polyethological disease, which can act as the only manifestation of various metabolic disorders, as well as a symptom of some serious internal pathologies. Calcinosis cutis has several varieties, which differ in clinical manifestations and the mechanism of salt deposition in tissues.
Causes
Usually, in most tissues of the body (with the exception of teeth and bones), calcium salts are in dissolved form – this allows them to be transferred with the blood flow to those organs where they are needed. However, in some cases, conditions may be created for their precipitation, which leads to the formation of pathological foci, including calcinosis cutis. Metastatic calcinosis is characterized by hypercalcemia, due to which increased amounts of calcium ions are carried throughout the body. At the same time, salt deposits are usually formed in many organs, but sometimes they can also occur in the skin. Such calcinosis cutis usually occurs against the background of malignant bone tumors, metabolic disorders, increased calcium intake with drinking water or food. A variant of this condition is also hypervitaminosis D, in which deposits of calcium salts can also occur in various tissues, including the skin.
The more common metabolic calcinosis cutis is caused by disorders in the skin and subcutaneous fat. At the same time, it is not necessary that there is a large amount of these salts in the body – due to various reasons (circulatory characteristics, pH level), with normal concentration of calcium in the blood, it still lingers in the tissues, leading to the development of the disease. Presumably, the delay of this element is produced by altered collagen fibers, which happens with various kinds of autoimmune and hereditary collagenoses. Another type of such a disease is the so-called secondary calcinosis cutis, which occurs at the site of scars, foci of various dermatological diseases (systemic scleroderma, dermatomyositis, neoplasms of the skin).
Symptoms
There are two main groups of skin calcification – metastatic and metabolic. Metastatic, as can be understood from the name, occurs due to the transfer of calcium salts from one part of the body to another. Their source may be a malignant tumor, a focus of osteolysis, affected kidneys and other organs. In this case, hypercalcemia occurs, and in those organs where the concentration of calcium ions exceeds their solubility under these conditions, foci of salt deposition form. Usually these are internal organs, tendons and muscle sheaths, sometimes subcutaneous fat and skin. With this type of calcinosis cutis, the symptoms are very erased and often hidden by the manifestations of the underlying disease. Individual nodules and seals on the skin and in the subcutaneous tissue can be determined, inflammatory manifestations and other pathological changes are usually not observed.
Metabolic calcinosis cutis is much more common and has several varieties characterized by different symptoms and manifestations. At the same time, hypercalcemia may not be observed, salt deposits in other tissues and organs may also not occur, since the formation of foci on the skin occurs due to a violation of local metabolism in the skin. Dermatologists distinguish the following forms of metabolic calcinosis cutis: limited, universal, tumor-like.
The pathogenesis of limited metabolic calcinosis cutis is reliably unknown, as are the causes of salt deposits in this case. At first, this pathology is completely asymptomatic – only sometimes a person can detect areas of compaction under unchanged skin. This type of calcinosis cutis mainly affects the upper extremities, the favorite localization of pathological foci is the areas above the projections of the joints (elbow, wrist, interphalangeal). Over time, the foci grow and become noticeable in the form of small nodules rising above the surface of the skin. Under the influence of injuries or spontaneously, they become inflamed, the skin above them turns red, their palpation becomes painful. This form of skin calcification mainly affects middle-aged and older women.
Universal calcinosis cutis is a more severe form of the disease caused by disorders of calcium and phosphorus metabolism. In this condition, pathological foci form on the extremities (also within the projection of large joints) and on the buttocks, occasionally a different localization may be observed. Salt deposits quickly acquire the character of dense nodules, but over time they begin to soften and turn into painless erosions and ulcers, the bottom of which is covered with white, easily crumbling contents. The healing of such injuries caused by calcinosis cutis occurs very slowly with the formation of noticeable scars and scars. This condition is most often diagnosed in children and young people under the age of 20.
Tumor–like calcinosis cutis is a rare form of the disease in which salts are deposited in the soft tissues of the joints and on the skin of the head. As a rule, the foci are solitary, can reach significant sizes (up to 10-12 centimeters), grow slowly and may resemble oleogranulomas outwardly. This form of calcinosis cutis is found almost only in children.
Secondary calcinosis cutis caused by pathological changes in some dermatological conditions, some specialists refer to the metabolic type, while others – to a separate form of this disease. Idiopathic calcinosis, which has an unclear etiology, is also singled out separately – it is assumed that it has signs of a hereditary disease. It was possible to reliably prove the genetic nature of skin calcification in relation to Teichlander syndrome, which is inherited autosomally recessive. In this condition, young people have calcifications in the subcutaneous tissue and soft tissues of the joints, all this is accompanied by fever and muscular dystrophy.
Diagnostics
Diagnosis of skin calcification in dermatology is made on the basis of the present status of the patient, X-ray examinations, histological examination of tissues at the site of pathological foci. A secondary role in the diagnosis is played by biochemical blood and urine tests, various examinations to search for the underlying pathology that could lead to a violation of mineral metabolism with such manifestations. On examination, foci of various localization and sizes are detected, depending on the type of calcinosis cutis and the stage of its course, most often these are nodules of rocky hardness in the projections of the joints, covered with unchanged or inflamed skin. Soreness occurs when inflammation is attached or a pathological nodule is injured, sometimes joint mobility may be impaired due to the significant size of calcinate. With universal metabolic calcinosis cutis in the resolution stage, painless ulcers with white contents on the bottom can be observed on the surface of the skin of the legs and buttocks.
When conducting an X-ray examination of a patient with calcinosis cutis, dense (sometimes denser than bone) shadows of irregular shape and consisting as if of separate small balls are determined at the site of pathological foci. In the case of old calcinates, the thin structure is no longer visible, only a dense focus is revealed. Sometimes on an X–ray you can see the presence of foci with calcium not only in the skin and subcutaneous tissue, but also in other tissues – around joints, tendons, some internal organs. Conducting a biopsy of such foci can sometimes be difficult, but if it was still possible to do it, then most often there will be perivascular deposits of calcium salts with degeneration of connective tissue fibers.
With metastatic calcinosis cutis, pronounced hypercalcemia will be determined in the results of a biochemical blood test, but in the case of a metabolic variant of the disease, this is not a mandatory manifestation. Idiopathic calcinosis, which has signs of a hereditary disease, is often manifested by phosphatemia. Also, the dermatologist can refer the patient to specialists of other profiles to search for pathology that could lead to the development of calcinosis cutis or prescribe additional tests. Often, a thorough survey and study of the patient’s anamnesis can help in the diagnosis of this condition, since it is possible to identify factors contributing to disorders of mineral metabolism. Differential diagnosis of skin calcification should be carried out with some forms of tuberculous and syphilitic lesions, skin neoplasms, gout and sarcoidosis.
Treatment
With relatively small sizes of calcinates, you can try to eliminate them in a conservative way – for this purpose, ammonium chloride and potassium iodide are used, which can normalize mineral metabolism and remove excess calcium from tissues. However, effective treatment with these drugs requires the use of high dosages, which dramatically increases the risk of side effects and increases the toxicity of these drugs. Therefore, it is necessary to calculate the course of treatment of skin calcification very carefully and strictly individually, based on a number of indicators of the body – the functioning of the excretory system, the number and size of calcinates, the presence or absence of concomitant diseases. Penicillamine is also used to treat this condition.
With significant calcifications or in the presence of limited joint mobility, surgical removal of pathological foci is used. However, this often does not eliminate the cause of calcinosis cutis, so it is very likely that new nodules will appear again on the site of removed salt deposits or on other parts of the body. Therefore, surgical intervention, laser removal or electrocoagulation must necessarily be performed against the background of conservative treatment. It is also important to limit the intake of calcium into the body – the doctor is developing a special diet with the inclusion of products with a reduced content of this element. According to the indications, the main disease is being treated, which provoked the development of calcinosis cutis.
Prognosis and prevention
Calcinosis cutis very rarely threatens the patient’s life, so the prognosis of the disease in this regard is relatively favorable. However, this indicator is strongly dependent on the factors that caused the disorder of mineral metabolism. So, with metastatic calcinosis cutis, it can be a malignant tumor, with secondary – systemic scleroderma and other serious and dangerous diseases. To prevent the development of such a condition, it is necessary to undergo a preventive medical examination in a timely manner (for earlier detection of dangerous diseases), not to self-medicate using calcium and vitamin D preparations. For persons prone to salt deposition in tissues, it is important to reduce the amount of calcium taken with food and water, periodically undergo examinations by a dermatologist and specialists of other profiles.