Among scleroderma, a focal form of this disease is distinguished. It is characterized by a less aggressive manifestation in comparison with the systemic form of this ailment. The focal type is accompanied by the appearance of white spots on the skin. To some extent, such spots may even resemble scars. These manifestations are distinguished by a favorable prognosis. Focal scleroderma is mainly detected in women. 75 percent of patients are women between the ages of forty and fifty-five. In recent years, focal scleroderma has become very common. In our article, we will talk about the causes, symptoms and methods of treating this phenomenon. And also consider how to prevent this disease. So, let's start with the reasons.
Reasons for the development of pathology
The exact causes of focal scleroderma today are not yet known to scientists. There are hypotheses that indicate the possible influence of a number of factors that contribute to the emergence of changes in the work of collagen. These include the following reasons:
- The appearance of autoimmune disorders leading to the production of antibodies that act against their own cells.
- The occurrence of neoplasms, the appearance of which in a number of situations is accompanied by focal scleroderma. Sometimes, such foci form several years before the appearance of the tumor.
- The presence of concomitant pathologies of connective tissues, for example, rheumatoid arthritis or lupus erythematosus.
- The patient has a genetic predisposition, since this disease is very often observed among relatives.
- Patient infections such as influenza, human papillomavirus, measles, streptococci and so on.
- The presence of disorders in the hormonal background in the form of menopause, pregnancy, due to abortion and so on.
- Excessive UV effect on the skin.
- Exposure to severe stress or choleric temperament.
- Getting traumatic brain injury.
Against the background of focal scleroderma, people observe excessive synthesis of collagen, which is responsible for skin elasticity. With this disease, due to its excessive amount, compaction occurs, and in addition, coarsening of the skin.
Pathology classification
To date, there is no single classification system for focal scleroderma. But experts often use the classification proposed by the scientist Dovzhansky. This system maximally displays all the clinical forms of this disease.
- Plaque type of focal scleroderma. This species is further divided into atrophic, superficial, nodular, deep, bullous and generalized types.
- The linear form of the disease is divided into ribbon-shaped and zosteriform.
- The next type is called white spot disease.
- Idiopathic atrophoderma is also distinguished.
- In addition, a disease is distinguished, against the background of which hematrophy of the face develops.
Is focal scleroderma dangerous to life? About it further.
Symptoms of the disease with plaque form
Among the clinical manifestations of the disease, the plaque form is most often found. In this case, an insignificant number of foci appears on the patient’s body, which usually pass through the following three phases: spots, the appearance of plaques and areas of atrophy.
First, several lilac spots appear on the skin at once, the sizes of which can be very different. After some time, a shiny and smooth area of the seal appears in the center of the spots, which has a yellowish color. Around this island, a pink border may remain. It is capable, in turn, of increasing in size. Actually, these signs judge the activity of scleroderma processes.
On the formed plaque, hair loss can occur, the processes of sweat and fat secretion cease, and the skin pattern disappears altogether. The skin area in this area cannot be taken with fingers in a crease. A similar appearance can persist for a long time, then the lesion, as a rule, undergoes atrophy.
Symptoms of a linear shape
This type of focal scleroderma (ICD-10 code - L94) is very rarely observed among adult patients, and is most often found in children. The difference from this species from the plaque type is only in the form of skin changes that look like stripes of white color and in most situations are located on the limbs or on the forehead.
What is dangerous focal scleroderma, interesting to many.
How is white spot disease manifested?
This type of focal scleroderma is often combined with its plaque type. Against the background of this variety, small grouped spots appear on the body of patients, which have a diameter of about one and a half centimeters. They can be located in different parts of the body, but very often localized on the trunk or on the neck. In women, similar lesions are observed in the labia.
Idiopathic atrophoderma and its symptoms
With idiopathic atrophoderma, scleroderma spots with irregular contours are usually located on the back. Their size is capable of reaching ten centimeters and even more. Idiopathic atrophoderma is observed, as a rule, in young women. The color of the spots is approaching a purple hue. Their center can sink a little and has a smooth surface, and a lilac ring may be along the contours of skin changes.
After the appearance of spots, signs of compaction of lesions for a long time may be absent. Sometimes such skin changes may be pigmented. Unlike the plaque type of focal scleroderma, this form is characterized by damage to the skin of the trunk, and not the face. In addition, rashes against a background of atrophoderma are usually not amenable to regression. They tend to progress gradually over several years.
Symptoms of facial hematrophy
This is a rather rare type of focal scleroderma, which is manifested by atrophic lesions of only a certain half of the face. This focus may be on the right or left. Skin tissue and adipose tissue undergo dystrophic changes. As for muscle fibers and bones of the facial skeleton, they are involved in pathological processes much less often or to a much lesser extent.
Hematrophy of the face is most often observed in women, and the onset of the disease, as a rule, occurs at the age of three to seventeen years. This pathological process can reach its activity by the age of twenty and most often continues until the age of forty. In the beginning, foci of changes in the bluish tint appear on the face. Then gradually they begin to condense and, as time passes, undergo an atrophic change, which is a cosmetic defect. The skin on the affected area of the face becomes wrinkled, and in addition, pigmented and thinned.
On the affected area of the face, hairs are usually completely absent, and the tissue lying under the skin lends itself to gross changes and deformations. As a result, the face acquires asymmetry. The bones of the facial skeleton, in turn, are also involved in this pathological process, especially if the onset of the disease occurred in early childhood.
Professional discussions
There is still debate among scientists regarding a possible connection between systemic and focal scleroderma. According to some experts, the systemic and focal type of ailment are varieties of the same pathological processes in the body. But other doctors believe that these diseases are significantly different. However, this opinion does not find precise confirmation, and statistical data indicate that in sixty percent of cases this form of scleroderma is transformed into a systemic type of pathology. According to various studies, the following factors contribute to the transition of focal scleroderma (ICD-10 - L94) into its systemic type:
- The onset of the disease before the age of twenty or after fifty years.
- The presence of a plaque or linear form of focal scleroderma.
- An increase in the number of anti-lymphocytic antibodies.
- An increase in coarse circulating immunity complexes.
- The severity of patients with dysimmunoglobulinemia, and in addition, a deficiency of the cell link in the immune system.
Pathology diagnostics
Diagnosis of focal scleroderma is problematic due to the similarity of its symptoms with many other diseases. In this regard, differential diagnosis is carried out with the following diseases:
- The presence of vitiligo or an undifferentiated form of leprosy.
- The presence of kraurosis of the vulva or keloid-like nevus.
- The presence of Schulman syndrome or a scleroderma-like form of basal cell carcinoma in a patient.
- The presence of psoriasis.
In addition, the following laboratory tests are usually prescribed to patients:
- Skin biopsy.
- Study of the Wasserman reaction.
- Conducting biochemistry and general blood analysis.
- Performing an immunogram.
A biopsy provides an opportunity to determine the diagnosis of the presence or absence of focal scleroderma with a 100% guarantee. This technique for this disease is the gold standard. Next, we learn how to treat focal scleroderma in modern conditions.
Disease treatment
Therapy of the disease should be comprehensive and lengthy. Against the background of the active course of pathology, the number of courses should be at least six. The interval between them should be from thirty to sixty days. With the stabilization of the progression of pathological foci, the interval between courses can be four months. Against the background of residual manifestations, treatment periods are repeated for preventive purposes once every six months. In this case, drugs should be included in the therapy, which are aimed at improving microcirculation in the skin. At the stage of the active course of pathology, the following drugs should be included in the treatment plan:
- The use of penicillin antibiotics in the form of drugs such as fusidic acid along with Amoxicillin, Ampicillin and Oxacillin.
- Treatment with antihistamines in the form of Pipolfen, Loratadin and Tavegil.
- The use of drugs to suppress excessive collagen synthesis. For these purposes, placenta extract is suitable along with Lidaza, Actinogial, Collalizin and Longidaza.
- Treatment with calcium ion antagonists. In this case we are talking about Verapamil, Phenigidin and Corinfar.
- The use of vascular agents, for example, nicotinic acid and preparations based on it: Trental along with Mildronat, Eskuzan, Berberin and Madecassol.
- Various vitamin-based creams may also be included in the treatment plan for focal scleroderma, and in addition, drugs such as Solcoseryl and Actovegin.
In the event that the patient reveals limited foci of the disease, treatment can be carried out with the appointment of phonophoresis with Ronidase or Lidase. For local therapy of focal scleroderma, it is necessary to apply ointment applications along with physiotherapeutic treatment. As local medicines, “Troxevasin” is usually used along with heparin, theonicol or butadione ointment. You should also be treated with "Heparoid", "Dimexidum", "Trypsin", "Chymotrypsin" or "Unitiol."
Still how to treat focal scleroderma?
"Lidase" is often used to perform electrophoresis. Ronidase is used for applications; for this, its powder must be applied to a napkin dipped in a special solution. Usually physiological saline is suitable for these purposes.
In addition to the above treatment methods, patients are recommended to perform the following sessions:
- A visit to magnetotherapy.
- Performing phonophoresis with Kuprenil.
- Conducting laser therapy.
- Visit vacuum decompression.
At the final stage of the treatment of the disease focal scleroderma, the main procedures can be supplemented with radon baths, and in addition, massage of the affected area. Recently, for the treatment of pathology, many experts recommend a decrease in the volume of drugs. They should be replaced with medicines that combine several required effects at once. These drugs include Wobenzym along with systemic polyenzymes.
As part of the treatment of focal scleroderma, a procedure such as hyperbaric oxygenation, which helps to saturate the tissues with oxygen, is often included in the general treatment plan. This method allows you to activate metabolic processes in the mitochondria. Thanks to this method, lipid oxidation is normalized, and in addition, it has an antimicrobial effect and improves blood microcirculation. Among other things, against the background of hyperbaric oxygenation, the regeneration of affected tissue is accelerated. This method of treatment is used by many experts, in addition, he has already repeatedly managed to prove his effectiveness.
Is focal scleroderma dangerous?
Local scleroderma does not pose a risk to life if the lesions are small and not widespread. If you find the first signs of the disease, you should consult a doctor. If the disease is detected on time, and the spread of the lesion is stopped by drug therapy, there is a high probability of a full recovery.
If untreated, scleroderma leads to major changes.
When the connective tissue grows significantly in the internal organs, various diseases develop: aneurysm of the heart, severe ischemia, up to necrotic damage to the internal organs due to a lack of oxygen and nutrients. Especially dangerous focal scleroderma in children.
The stronger the heart is involved, the sooner the death will occur.
It also depends on the nature and variety of whether focal scleroderma is dangerous to life. Some types of illness are more acute and faster than others.
Pathology Prevention
In order not to expose the body to such diseases once again, simple rules should be followed:
- Need to wear loose shoes.
- It is very important to dress warmly when it is cold outside.
- You can not be under air conditioning for a long time.
- Drink less coffee, which helps to narrow the vessels.
Focal scleroderma. Reviews
Reviews about this pathology are abundant. If the disease was observed in relatives, then the person is at risk of encountering an ailment. In this situation, precautions cannot be ignored. Also, reviews confirm that it is not easy to cure the disease. You need to eat right, avoiding nervous tension and excessive tanning. A full rest along with a normalized daily routine are the key to health, and this, of course, will help protect yourself from such a disease.
We examined the medical history of focal scleroderma.