The disease occurs in the tissues accumulation of fat cells and their growing, called mastocytosis. Such a phenomenon may have different forms and depending on this, experts determine measures required patient For help.
The manifestations of pathology are somewhat different in patients children's age And in adults.
According to the ICD-10, mastocytosis has the following code: Q82.2.
The disease occurs heavier than the kind of more children suffer. Systemic mastocytosis is defeated by internal organs.
Mastocytosis in children (photo)
According to the manifestations of illness and age of sick people distinguish such forms:
The video below will tell what masocytosis (pigment urticaria) of the skin:
Skin lesions are classified in this way:
During the skin form of the disease, such signs are characteristic:
If there is a systemic defeat, this means that symptoms that are characteristic of skin form can appear. This is possible if systemic disorders are combined with the skin form of the disease.
Symptoms that are inherent in a system form:
Medical science has not yet solved why this disease appears in a small number of people. There are assumptions that pathology is associated with genetic predisposition. However, in practice, the theory does not find full confirmation, because the predispositions do not detect half of the sick people.
About the symptoms that have skin and systematic mastocyitosis, read below.
This video will tell about home therapy of mastocytosis, who will tell who and how he cured:
To obtain assistance in mastocytosis, contact the dermatologist. Self-treatment is unacceptable. Children's mastocytosis is capable of passing himself.
The appointment of a doctor takes into account the defeats that the patient received. A specialist prescribes a sick symptomatic treatment.
To help precisely at the same time, a technique has not been developed today.
Physiotherapy procedures can be assigned to the treatment of manifestations of mastocytosis, namely, the use of ultraviolet irradiation.
To facilitate well-being during problems on the skin, a specialist may appoint an application (externally) corticosteroid drugs.
In cases and systemic lesions, antihistamines are used and skin option.
Operational intervention is appointed by a specialist in cases where education on the skin has increased to large sizes.
With systemic defeat, the removal of organs is sometimes prescribed if experts find it necessary.
To eliminate the provocation of the disease, it is necessary to remove factors that contribute to the increase in fat cells.
Systemic skin damage can lead to serious damage to organs. This situation significantly affects their ability to perform their functions.
Ultrasound in the disease Mastocytosis is shown in this video:
Mastocytosis, or pigment urticaria, refers to a rather rare and not yet very well-studied disease. It can be overtaken with the same probability of both men and women. More often occurs in children than in adults - about 75% of cases. Many confuse this ailment with a gloomy or consider one of its forms. This opinion is erroneous. There is practically no links between diseases. The coincidence in the title can be considered an accident. The only one - chronic urticaria in some cases can provoke the development of mastocytosis. About other reasons for illness, as well as its specifics, symptoms and treatment, read in this article.
Video - Mastocytosis: the seriousness of the disease and possible treatment
Mastocytosis is a disease blood system. It is characterized by education, mass reproduction and accumulation of special cells in the body. The latter are called mastocytes or still fat cells that play an important role in the functioning of the protective system, being an integral part. This gives medical nurses to assume that the pigment urticaria can become a consequence of immunity problems. Accumulating in living fabrics, impregnate the skin and internal organs, mastocytes provoke the occurrence of various symptoms characteristic of this disease.
Two main forms of mastocytosis are distinguished: skin and systemic. They have various symptomsThe causes of the occurrence, as well as projections of treatment.
Mastocytosis in children, as a rule, is skin. Develops in early age. The main risk group is kids up to two years (often diagnosed in newborns). Pathological processes affect the skin of the body. There are no lesions of the internal organs.
Cases of transition of this form in a more complex systemic single. Most patients forget about the disease to adolescence, and the most often spontaneous cure. Sometimes the skin is found in adults. Undesses in such cases are not cured, but does not progress. In addition to the skin, internal organs affect.
Mastocytosis in adults, as well as children from 13-14 years old, usually has a system form. It is affected by it, mostly fabric internal organs. The epidermis in some cases is also affected, but this is not a specific manifestation. Systemic mastocytosis proceeds more difficult and the inclination to self-esteem is no different. The disease is considered chronic.
In the most unfavorable cases, pathology passes into a malignant shape, which is called torching leukemia. This is a very aggressive view of cancer with an unfavorable forecast. Cure him extremely hard.
In the mastocytosis of the skin shape (and sometimes system) distinguish five types of lesion of the epidermis.
The reasons for pigment urticaria (mastocytosis) are still considered unclear. There is a version of hereditary prerequisites. In her favor, it is evidenced by the fact that mastocytosis often detects from relatives. But on the other hand, approximately 50 percent of cases of gene predisposition is not fixed.
Contemporary science believes that the pigment urticaria may have an immune or not immune nature. The disease belonging to the second type is often developing under the influence of the following factors:
With skin mastocytosis and systemic symptomatic picture is different. For the first form characteristic:
Symptoms of systemic mastocytosis are:
Some people, finding bumps and blisters on their skin, take them for manifestations of urticaria and try to treat this disease independently. The diagnosis they actually put on the photo from the Internet, and the drugs find there. For example, they buy wide advertised drugs brand "Transfer factor", which are really effective in conventional urticaria, but with pigment, they are not always acquitted.
Such behavior is extremely irresponsible in relation to their own health. If there is an alarming signs, you need to contact the doctor and pass the survey. In order to diagnose the illness, blood tests are usually prescribed, which show the presence or absence of fat cells; A chromosomal study is carried out, as well as skin biopsy. As additional methods often make x-rays and MRI bones. To identify the degree of damage to organs with the inner form of pathology, the liver, spleen, etc. In mandatory, the urine is investigated.
Treatment of mastocytosis refers to the competence of therapists and hematologists. Therapy is complex. As a rule, drugs are prescribed, which reduce the amount of serotonin, which have anti-inflammatory and anti-allergic effect. Also, most likely, cytostatic and corticosteroids will have to take.
Cure pigment urticule (especially systemic type) is very difficult. In many cases, therapy is only symptomatic; In addition, the patient is prescribed immunomodulators like "Transfer factor" or others. Sometimes you have to resort to surgical interference by which the neoplasms are removed.
In order not to exacerbate the situation to people suffering from mastocytosis, it is strongly recommended to abuse sunny baths, saunas, baths and solariums. This may be extremely negatively affecting the body's condition. Persons having a hereditary predisposition should be especially careful about their health.
Suffering from ordinary urticaria should not allow its transition to chronic form. And here very relevant becomes just preparations "Transfer factor". In their composition only natural componentsThey passed clinical trials and well established themselves in the treatment of urticaria. In addition, "Transfer factor" is one of the most effective tools In terms of strengthening immunity.
As noted above, the skin mastocytosis in children as a rule passes to adolescent age. But parents, finding characteristic spots from the baby, often fall into panic. The famous Children's doctor Evgeny Komarovsky calls on them not to do this, but not to do with self-medication. Choo must need to show the doctor who will prescribe adequate therapy. Much worse, according to Komarovsky, if there is a common urticaria, threatening swelling. This condition is considered life-threatening. The doctor's personal site has a whole branch of the forum dedicated to Mastocytosis, where parents discuss the features of the disease and the treatment of their children.
So, a pigment wretched is a pathology, which in some cases can be completely non-hazardous and passed independently, and in others it is capable of significantly reduce the quality of life and even provoke oncology.
In order to maximize themselves to protect yourself from negative consequences, it is not necessary to engage in self-medication, taking the "transfer factor" or anything else. It is important to put an accurate diagnosis and get the appointment of a competent specialist. Then you can hope for the most favorable forecast and achieve if not full cure, then lifelong remission of mastocytosis.
Mastocytosis is a disease of the circulatory system, in which massive special cells are formed - Mastocytes, Labrocytes and tissue basophiles. These cells are impregnated with different internal organs and skin.
Mastocytosis has two forms - skin and systemic.
Skin mastocytosis is, as a rule, mastocytosis in children. When mastocytosis, children appear pigment urticaria, diffuse or local maculophaulous rash on the skin of a brown or orange-pink color, which occurs due to the accumulation of a large number of small fat cells.
It is less common to diffuse skin mastocyitosis, in which the infiltration of the skin of the skin with obese cells without lesions is discrete.
Most often, skin mastocytosis in children appears under the age of two years.
The system form of mastocytosis is often occurring in adults. Manifests in the form of multifocal lesions bone marrow, it can also affect the skin, the lymph nodes, spleen, liver and gastrointestinal.
There are several classifications of system mastocytosis:
Painless. Has a positive forecast;
Associated with other hematological disorders (with myeloproliferative disorders, lymphoma, myelodysplazia);
Aggressive mastocytosis. Characterized by serious organ dysfunction;
Touchless leukemia, in which more than 20% of fat cells are observed in the bone marrow, there are no skin lesions, multi-Russian lesions. It has a bad forecast.
Skin mastocytosis mainly affects only skin cover. At the same time, the following symptoms are observed:
Redness of the skin;
Skin itch;
Reduced pressure;
Periodic increase in heartbeat;
Periodic increase in body temperature.
Systemic mastocyitosis affects:
Nervous fabric;
Spleen (it increases in size);
Bone marrow (normal cells in the bone marrow are replaced by mastocytes, leukemia is formed);
The liver (the liver increases, is compacted, and fibrous nodes appear in it);
Digestive tract (diarrhea and peptic lesions appear);
Bone system (osteoporosis (softening of bones) and osteosclerosis (bone tissue is replaced by connecting), bone pain appear);
Lymph nodes (increasing and appearance of painful sensations).
For the diagnosis of mastocytosis, blood is examined .Oe Mastocytosis is evidenced by the presence of a plurality of fat cells, as well as triptions and histamine, which are products of their livelihoods.
To determine the quantity, type and degree of development of fat cells, biopsy of the skin, bone marrow and other affected organs is carried out.
To identify any disorders in the spleen or liver, kg and ultrasound are carried out. During the lesions of the digestive tract, endoscopy is applied.
To begin treatment of mastocytosis, contact the therapist and hematologist.
For the treatment of mastocytosis, symptomatic therapy is applied, which is aimed at suppressing the production of fat cells of biologically active substances:
Underwasil sodium, ketotifen - to stabilize membranes on fat cells;
Zoda, Tueva, Supratin - as antihistamine.
With diffuse systemic mastocytosis, the removal of the spleen is shown.
With severe form of mastocytosis or during its resistance to antiallergic drugs, the PuU-therapy is used. This therapy effectively reduces the amount of rashes. Improvement is observed after 25 PuU-therapy sessions of 3-5J / cm² per session.
Video from YouTube on the subject of Articles:
Mastocytosis (Touchless leukemia) - systemic disease, a morphological substrate of which is massive infiltration of the skin, various organs and tissues with fat cells (masculocytes, labrocytes, tissue basophilies). The disease is rare, its frequency does not depend on the floor. It is usually developing during the first two years of the child's life (75% of cases) and in half cases are spontaneously permitted to a publity period. The systemic mastocytosis is more often sick of adults.
Etiology and pathogenesis of mastocytosis Unknown. Sometimes it is transmitted to an autosomal dominant path, although in most cases the patients do not have family anamnesis.
Histological picture of all forms of mastocytosis Manifested by infiltrates consisting mainly of fat cells. The metacromazis of the granules of the fat cells is detected using toluidine, methylene blue, as well as Naphol-AS-D-chlorocetate Esterase (LEDER method), staining cytoplasmic granules of fat cells in red. With maculopapulous and tele-angitatatic types of mastocytosis, obese cells are located mainly in the upper third of the dermis around the capillaries. The nuclei of the fat cells is more often spin-like, less often round or oval. Due to the fact that the fat cells may be present in a small amount and on sections painted with hematoxylin and eosin, their kernels resemble the cores of fibroblasts or pericitis, special color is required to establish the correct diagnosis. For multiple nodes or plaques, as well as with large solitar nodes, the fat cells form large clusters by type of tumor, infiltrating the entire dermis and subcutaneous fatty tissue. When the fat cells lie in dense clusters, their kernels are rather cubic than spindle-shaped, have eosinophilic cytoplasm and well-defined cell boundaries. This gives them a rather characteristic view, so the diagnosis can be put even without special staining. With a diffuse (erythrodermic) type in the upper part of the dermis, dense tanning infiltrates from fat cells with rounded or oval nuclei and well-defined cytoplasm are observed. With all types of mastocytosis (with the exception of teleangectic), eosinophils may occur among the obese cells. Increasing the amount of eosinophils and extracellular granules of fat cells in a bioptate taken after friction of the focus, indicates the release of the granules from the fat cells. The character of bubbles with subepidermal mastocytosis; However, due to the regeneration of the epidermis at the base of bubbles, the bubbles can be localized intapidermally. The bubble often contains fat cells, as well as eosinophils. Pigmentation of elements with mastocytosis is due to an increase in the number of melanin in the cells of the basal layer of the epidermis, less often - the presence of melanophages of the upper part of the dermis. With systematic mastocytosis, along with the lesion of the skin, paratrabecular accumulations of fat cells in the bone marrow, diffuse infiltration of the red pulp spleen, peripollicular infiltration of the peripolicular and paractic infiltration of the peripollicular and parakortical zones of lymphatic nodes are detected. The accumulations of the fat cells are observed in the heart, kidneys, mucous membrane of the gastrointestinal tract.
Allocate 4. forms of mastocytosis:
More than half of the patients mastocytosis Complaints are limited to lesion of the skin. About 1/3 patients complain about itching, attacks of redness of the skin, paroxysmal tachycardia, falling arterial pressure, periodic increase in body temperature and so on. These symptoms are caused by the degranulation of obese cells and are observed with common forms of skin mastocytosis or internal organs.
With mastopitosis There are five types of skin lesions (and the first two of them are observed both in babies and in adults).
Maculopapulous type of mastocytosisrepresented by dozens or even hundreds of small red-brown hyperpigmented spots and papules, which, after friction (Darya-Unny), acquire ultrasound-like, is the most frequent.
Multiple nodal type of mastocytosis It is represented by multiple dense semi-shaped pink, red or yellow diameters, a diameter of 0.5-1 cm with a smooth surface, sometimes nodes merge into plaques, the phenomenon of Darya-Unny is poorly expressed, especially in adults.
Type of large solitary nodes, or mastocytes, is manifested by a node with a diameter of 2-5 cm. having a smooth or crumpled, like an orange peel, surface and rubber consistency. Occasionally meets up to 3-4 knots. Nodes arise exclusively in babies, a cup in the neck, torso, forearm. Test Darya-Unny positive. Traumatization leads to the formation of bubbles or bubbles on the surface of the node, and the feeling of tingling. Mastocytes are usually regressing spontaneously, while they are wade and smoothed. In some cases, the nodes are associated with rashes characteristic of maculopapulous type of mastocytosis.
Diffuse (erythrodermic) type of mastocytosis It always begins in early childhood. It is characterized by large mercenary foci of yellow-brown infiltration, usually in the axillary depressures and inter-fodded folds. Focis have an irregular shape, clear boundaries, dense (to woody) consistency, on their surface it is easily an ulceration, cracks, excoring. The progression of the pathological process can lead to erythrodermia. At the same time, skin covers acquire a test or dense consistency, their color varies from pink-red with yellow-brown tint to dark brown. Darya-Unny test is positive, and even easy injury to the lesion leads to the occurrence of bubbles, often accompanied by an intense itch. When prevailing in a clinical picture of bubbles, they talk about bullous mastocytosis. The course of the disease is gradually improved, progression into systemic mastocytosis occurs rarely, death occurs only in babies as a result of histamine shock.
Teleangiectic type of mastocytosis (Type of Patty Teleangectasia) - a rare form of mastocytosis, is found primarily in adults, mostly in women. It is manifested by common cornched spots of various outlines consisting of bright teleangectasia. Filmed on a pigmented background. A small tendency to the appearance of blisters in friction places or spontaneously is noted. Breast leather and limbs are amazed. Characterized itching. In some cases, bones and peptic ulcer arise.
System Mastocytosis It is characterized by the damage to the internal organs in combination with skin mastocytosis or without it, it is found equally often in men and women. It is observed in 10% of patients with skin manifestations of mastocytosis. At the same time, in most cases, skin lesions are preceded by signs of the system process. Skin changes in systemic mastocytosis are represented by maculopapulous, multiple noded or diffuse (erythrodermic) types.
In the process of mastocytosis Any organs and systems can be involved. Most frequently bones, liver, spleen, lymph nodes, gastrointestinal tract, CNS. 20-30% of patients with mastocytosis have bone damage, which, with x-ray, are manifested by osteoporosis and osteosclerosis. Osteolatic changes are accompanied by pain syndrome. About 1/4 bone lesions are masculosites, but they are non-specific in children. Liver lesions are manifested by asymptomic touched infiltrates and fibrosis with hepatomegaly and diffuse mastocytosis.
System Mastocytosisfor flow can be benign and malignant. The malignant mastocytosis includes touched leukemia, a flat-body sarcoma, options for systemic mastocytosis associated with lymphomas, solid neoplasms or malignant diseases blood.
Touchless leukemia is manifested by basophilia, neutrophilia with a significant shift of the kernel to the left, hepato and splenomegaly, a slight hyperplasia of lymph nodes. In the bone cerebral point, the foci of blass fat cells are found.
Diagnosis of skin mastocytosis Installed on the ground clinical picture, Positive Darya-Unny test, increasing the fat cells in the lesions foci. However, it should be borne in mind that it may be absent with the Mastocytian and, if the lesion is injected in injury 2-3 days before the study (the time required to restore the granules of fat cells - 2 -3 days).
In the diagnosis of mastocytosis The definition of histamine in the urine (histaminuria) is used, but its level also rises with inflammatory, allergic diseases, as well as in healthy individuals (for example, athletes). To eliminate systemic mastocytosis, the main metabolite of histamine - 1-methyl-4-imidazoluxous acid is determined, the content of which correlates with the activity of severe proliferation and sharply rises with systemic mastocytosis, while under the skin forms its level is close to normal.
It is carried out with pigment nevys, freckles, lenti-ginosis, from which mastocytosis is distinguished by the presence of a positive phenomenon Dari-Unny. Papulse and nodal elements of mastocytosis are distinguished from xanthog, dematofibrome, histiocytosis X, non-lawnotendothelioma based on histological research data. Unlike medicinal toxidermia, there is no connection between the disease with the reception of drugs during mastocytosis. With insect bite, epidermal bubble newborn, multiform exudative erythema differential diagnosis Performed on the basis of clinical data and results of urine research on histamine. Mastocytosis It is difficult to distinguish between the congenital cane-calormium of the Rothmund-Thomson of an autosomal-recessive disease, clinically manifested in the first years of life and characterized by erythematous stains, replacing mesh hyperpigmentation, atrophy and tele-angioctasis on the skin of the face, neck, less often on the limbs. Unlike mastocytosis, low growth, microcephaly, teeth dystrophy, hair and nails, cataract, mental retardation are noted. Teleangiectic form of mastocytosis differs from atrophic vascular pyaculodermia of Jacobi, characterized by focal hyper- and hyperopigmentation, teleangectasis and skin atrophy, generated by the nature of the process, the presence of fifthly papular rashes, prone to merge, with the formation of large foci of lesion, the possible presence of common symptoms (attacks "tides ", Strengthening itching, tachycardia, etc.) or lesions of the internal organs. Pigment purple and lichenoid angioderotitis Geuztero-Blum, unlike mastocytosis, is striking mostly men of middle age and is characterized by brilliant lihenoid papulas, hemorrhagically pigmented spots in area lower extremities, as well as the negative phenomenon of Dari-Unny.
Differential diagnosis of mastocytosis It is also carried out with diseases accompanied by increasing the number of fat cells in histological preparations. Touchless proliferates take place with a jet mastocytopia, with hemangioma, dermatophibrome, lymphoma, lymphogranulomatosis, etc. An important feature of the histological diagnosis in mastocytosis is that in the "old" focus of mastocytosis, obese cells can be in a small amount. In this regard, the most reliably in the histological picture can be diagnosed with mastocytomas having an unpretentious structure. In other cases, the diagnosis is established on the basis of histological research data based on clinical data.
Treatment of Mastocytosis Not enough effectively. Isolated mastocytes are removed surgically. Cyprogeptadine (periactin) chlorohydrate is used for the treatment of mastocytosis (periactin) of 16 mg / day, H2-blockers of histamine, serotonin inhibitors. Preparations that hinder the degranulation of mastocytes (shitty, etc.), cytostatic (recipidin, spirobromine), therapy. With itching and "tides" apply antihistamines. With bullous forms, corticosteroid hormones are prescribed.
Prevention of mastocytosis It is to prevent the impact on the body of activators of the degranulation of fat cells, such as colds, the effects of cold, heat, injury, insolation, medications (especially morphino-opium group of alkaloids and acetylsalicyl cysts).
Mastocytosis is a complex systemic disease, which is due to the accumulation and proliferation in the tissues of the body of obese cells. This disease (the malignant shape of which is called the touched leukemia) was first described in 1869, as a chronic urticaria, leaving after herself brown stains. The term was introduced only in 1953. This is a rare disease that is much more common in children, and, as a rule, is limited to lesion of the skin, while adults almost always develops a system form.
Most often, mastocytosis strikes the skin of children, but also can affect other organs, such as intestines, stomach, spleen, liver, bones or lymph nodes. Heavy cases of tinnitus leukemia are accompanied by such severe blood diseases as acute leukemia, chronic neurosis or lymphoma. Approximately 90% of patients suffer from a pigment wretched. Treatment is most often consigned to the appointment of various antihistamine drugs and control over the main disease.
The etiology of the disease is unknown. Purchase cells are distributed in almost all organs and are located in close proximity to the lymphatic and blood vessels, epithelial surfaces and peripheral nerves, which allows them to perform protective and regulatory functions, as well as participate in various inflammatory reactions.
The pathogenesis of the disease is based on the release of various mediators of obese cells, including heparin, histamine, leukotrienes and cytokines of inflammation. Extensive infiltration by body cells, leads to violations in its functions. Among the reasons that are able to launch mediators are noted by heavy physical exercise, action different toxic substances Chemical or organic origin. Also in the etiology of the disease there is a mutation of genes that encode the tyrosine kinase receptor. There is a theory on the transmission of the disease with an autosomal dominant path, although most of the patients have no family anamnesis.
Based clinical features, distinguish four forms of the disease:
During the skin, the disease is mainly affected by the skin and there are such basic symptoms as skin itch, seascapes of heartbeat, red skin, pressure reduction and periodic body temperature increase.
Skin mastocytosis is characterized by five main types of skin lesions:
With systematic mastocytosis, various internal organs are infiltrated by obese cells. At the same time, in the work of the organs affected by disease, there are multiple specific disorders - bone pain, sealing and increasing liver, diarrhea, peptic lesions of the digestive tract, an increase in the spleen, the formation of leukemia, an increase in lymph nodes, damage to the nervous tissue.
The basis of the diagnosis of mastocytosis is clinical manifestations Diseases and detection on the skin and tissues of a large number of fat cells. Apply a complex of laboratory and instrumental methods of research - blood tests, biopsy of the skin, affected organs and bone marrow, chromosomal analysis to determine anomalies, radiographic examination of bones, MRI, ultrasound and CT internal organs, endoscopic diagnostic methods.
If mastocytosis is not accompanied by any system symptoms, it does not need treatment, since it has a magnificent tendency to self-esteem before adolescence. Hematologists and therapists, no specific methods of therapy are engaged in the disease, therefore, symptomatic therapy is used. Treatment is mainly based on the use of antihistamine preparations, which suppress the scaffolding of biologically active substances. With complex forms and severe course The disease is shown the use of PuU-therapy. Operational intervention in the form of removal of the spleen is required during system diffuse form. The forecast for the treatment of mastocytosis is determined by its form and degree of damage to the internal organs.