Is Schamberg's disease a cosmetic defect or a serious disease? Hemosiderosis: symptoms and treatment Hemosiderosis of the skin of the legs treatment with folk remedies

Hemosiderosis is a disease belonging to the group of pigmentary dystrophies. It is characterized by the accumulation of excess amounts of hemosiderin, an iron-containing pigment, in the tissues of the body.

Skin manifestations of hemosiderosis

Causes and risk factors

Hemosiderin is formed during the breakdown of hemoglobin molecules under the action of certain enzymes in sideroblast cells. Normally, a small amount of hemosiderin is contained in the cells of lymphoid tissue, bone marrow, spleen and liver. With an increase in hemosiderin synthesis, its excess is deposited in the cells of other tissues.

General hemosiderosis is always a secondary pathology, the development of which is caused by autoimmune processes, intoxications, infections or blood diseases. Most common reasons hemosiderosis are:

hemolytic anemia;
poisoning with hemolytic poison (quinine, lead, sulfonamides);
leukemia;
multiple blood transfusions;
cirrhosis of the liver;
Rhesus conflict;
infectious diseases ( relapsing fever, malaria, brucellosis, sepsis);
decompensated heart failure;
chronic venous insufficiency;
diabetes;
dermatitis;
eczema.

Some experts believe that hereditary predisposition and immune system disorders play an important role in the pathological mechanism of hemosiderosis development.

Forms of the disease

According to the degree of spread of the pathological process, the following are distinguished:

general (generalized) hemosiderosis - it is caused by intravascular (endovascular) hemolysis of red blood cells that occurs against the background of any systemic processes. Hemosiderin is deposited in the spleen, red bone marrow, and liver, as a result of which they acquire a rusty, brown color;
local (local) hemosiderosis - develops as a result of extravascular (extravascular) hemolysis. The accumulation of hemosiderin can be observed both in small areas of the body (in the area of hematomas) and within an organ (for example, the lung).

According to the characteristics of the clinical course, the following are distinguished:

hemosiderosis of the skin (ocher dermatitis, purpuric angiodermatitis, lichenoid pigmentary dermatitis, senile hemosiderosis, annular telangiectatic purpura of Majocchi, Schamberg's disease);
pulmonary idiopathic hemosiderosis (brown induration of the lungs).

Pulmonary hemosiderosis progresses and leads to disability in patients. It can be complicated by massive pulmonary hemorrhages, leading to rapid death.

In some cases, hemosiderin deposition causes dysfunction of the affected organ and the development of structural changes in it. This form of hemosiderosis is called hemochromatosis.

Symptoms

The clinical picture of hemosiderosis depends on the form of the disease.

Pulmonary idiopathic hemosiderosis is diagnosed mainly in children and individuals young, is characterized by periodically occurring pulmonary hemorrhages of varying severity, increasing respiratory failure and persistent hypochromic anemia.

IN acute phase diseases are observed:

dyspnea;
chest pain;
cough with bloody sputum;
pale skin;
icterus sclera;
cyanosis of the nasolabial triangle;
weakness;
dizziness;
increase in body temperature.

During remission clinical manifestations diseases are very weakly expressed or completely absent. After each period of exacerbation of idiopathic pulmonary hemosiderosis, the duration of the remission period decreases.

The cutaneous form of hemosiderosis occurs more often in men over 40 years of age. The course is chronic and benign. Brick-red pigment spots appear on the skin of the forearms, hands, legs and ankles. Over time, the color of the rash elements changes to yellowish, dark brown or brown. Defeats internal organs with the cutaneous form of hemosiderosis does not occur, general state doesn't suffer.

Diagnostics

Diagnosis of hemosiderosis is based on the characteristic clinical signs of the disease, data from laboratory and instrumental examination methods. Appointed general analysis blood, serum iron concentration is determined.

If hemosiderosis is suspected, a desferal test is performed. To do this, deferoxamine is injected intramuscularly, and then the iron content in the urine is determined. The test is considered positive if a portion of urine contains more than 1 mg of iron.

To confirm the diagnosis, a puncture biopsy of the bone marrow, lung, liver or skin is performed, followed by histological examination of the resulting tissue.

General hemosiderosis is always a secondary pathology, the development of which is caused by autoimmune processes, intoxications, infections or blood diseases.

For hemosiderosis of the lungs, the following is also carried out:

spirometry;
microscopic examination sputum;
lung perfusion scintigraphy;
chest x-ray;
bronchoscopy.

Treatment

Therapy for pulmonary hemosiderosis begins with the administration of corticosteroids. If it is ineffective, patients are prescribed immunosuppressants and undergo plasmapheresis sessions. In some cases, it is possible to achieve stable remission after removal of the spleen (splenectomy). In addition, there is symptomatic therapy with the prescription of bronchodilators, hemostatics and other drugs (depending on the manifestations of the disease).

Treatment of cutaneous hemosiderosis consists of local application corticosteroid ointments. Angioprotectors, calcium preparations, rutin, and ascorbic acid are also prescribed. In severe cases of the disease, the use of deferoxamine and PUVA therapy is indicated.

Possible complications and consequences

The most common complications of pulmonary hemosiderosis:

recurrent pneumothorax;
pulmonary hemorrhage;
pulmonary infarction;
expansion and hypertrophy of the right heart;
pulmonary hypertension;
chronic respiratory failure.

Forecast

The prognosis for pulmonary hemosiderosis is serious. The disease progresses and leads to disability in patients. It can be complicated by massive pulmonary hemorrhages, leading to rapid death.

With the cutaneous form of hemosiderosis, the prognosis is favorable.

Pulmonary idiopathic hemosiderosis is diagnosed mainly in children and young people.

Prevention

Prevention of disorders of hemosiderin metabolic processes consists in timely treatment hematological, vascular and infectious diseases, prevention of chemical or drug intoxication, blood transfusion complications.

HEMOSIDEROSIS (haemosiderosis; Greek, haima blood + sider iron + -osis) - excessive formation of hemosiderin and its deposition in tissues, observed in a number of diseases and caused by for various reasons. Among them, there are endogenous ones, for example, massive destruction of red blood cells, increased absorption of iron in the intestine, and exogenous ones, for example, with frequent blood transfusions. G. can be local or general in nature.

The body has a fairly constant normal conditions iron reserve in the form of two protein complexes - ferritin and hemosiderin. Ferritin is a water-soluble compound, the molecule of which consists of a protein part (apoferritin) and colloidal strands of ferric iron. Hemosiderin is a water-insoluble yellow-brown pigment; its protein part is identical to apoferritin. Hemosiderin, unlike ferritin, also contains inorganic compounds phosphorus, sulfur, nitrogen and non-protein organic substances. There is an idea about the transformation of ferritin into hemosiderin [Shoden, Stegeron (A. Shoden, P. Sturgeron)].

It has been established that iron levels are maintained by the regulation of absorption, and not by the process of active excretion. Every day, 0.5 mg of iron is excreted in feces (by cells of the exfoliating intestinal epithelium), 0.2 mg in urine, and 0.5-1.5 mg in women during menstruation. These small losses are compensated by iron absorption. Iron absorption occurs mainly through the mucous membrane of the duodenum.

IN food products iron is contained in quantities many times greater than the need for it. However, the amount of intestinal absorption of iron depends little on its content in the products entering the intestine.

Conrad, Weintraub, Crosby (M.E. Conrad, L.R. Weintraub, W.H. Crosby) using radioactive iron showed that iron absorption occurs in two phases: the entry of iron from the intestinal lumen into the mucous membrane and the movement of iron from the mucous membrane into the blood plasma and fabrics. According to M. S. Wheby's hypothesis, iron absorption consists of three phases: penetration into the mucous membrane from the intestinal lumen, penetration from the intestinal mucosa into the plasma, and iron deposition in the mucous membrane. Penetration of iron into the mucous membrane from the intestinal lumen occurs faster than from the mucous membrane into the plasma. However, when the body's need for iron increases, its entry into the plasma accelerates and iron in this case is not deposited in the form of a reserve.

It has been shown that 80% of the iron adsorbed by the intestinal mucosa quickly passes into the plasma and combines with the transport protein transferrin. Next, the iron bound to transferrin is taken up by red blood cell precursors and used again for the synthesis of hemoglobin. Weintraub (1965) was able to identify the dependence of the intensity of intestinal absorption of iron on the level of hemoglobinogenesis, but the mechanism of the quantitative relationship between these two processes remains unclear. The remaining 20% of absorbed iron is fixed in the tissue by iron-sensing protein. If the body's need for iron increases, this bond weakens and the iron passes into the plasma, and the receptor protein again fixes the iron with a new intake of food. If the body's iron requirement is low, then most of the adsorbed metal is fixed in the form of hemosiderin and ferritin in intestinal cells. Since the latter quickly desquamate (usually after 3-4 days), the iron fixed in them is released into the intestinal lumen.

If there is an abnormality in iron absorption, such as, for example, with hemochromatosis (see), hemosiderin is deposited in the organs. The pathogenesis of these disorders is not completely clear; It is indisputable, however, that in the intestine the absorption of iron does not depend on its reserves in the body.

Rice. 24. Accumulation of hemosiderin ( of blue color) in the epithelium of convoluted tubules of the kidneys with secondary hemosiderosis (Perls stain; x 120). Rice. 25. Hemosiderosis of the liver (hemosiderin - blue; Perls stain): a - in liver and Kupffer cells (X 400); b - diffuse hemosiderosis in thalassemia (X100). Rice. 26. Alveolar macrophages with hemosiderin (brown) in essential pulmonary hemosiderosis (X400).

Hemoglobin of destroyed erythrocytes is fixed by reticular, endothelial and histiocytic cells of the spleen, liver, kidneys (color. Fig. 24 and 25), bone marrow, lymph nodes, where it is preformed into hemosiderin grains, causing these organs to become rusty. Brown.

Methods and techniques for detecting hemosiderosis

To assess iron reserves, complexones are used, which bind and remove ferric iron from the body. The most commonly used complexon is desferal (syn. deferoxamine), which complexes with the iron of iron-containing proteins - ferritin and hemosiderin (but not with the iron of hemoglobin and iron-containing enzymes). It forms a stable complex with ferric iron and is excreted by the kidneys in the form of ferrioxyamine (see Complexons). The introduction of desferal does not significantly affect the release of other metals and trace elements.

Desferal test: 500 mg of the drug is administered intramuscularly to the patient once and for 6-24 hours. After the injection, urine is collected and the amount of iron is determined. The test is considered positive if the iron content in the urine is more than 1 mg. Based on the data from the desferal test, it is possible to decide on the need for complexone therapy to remove excess iron from the body, as well as the advisability of treatment with iron preparations when iron reserves are depleted. The desferal test is used as a diagnostic test in patients with thalassemia; This test can be used to exclude iron deficiency anemia(a common misdiagnosis in thalassemia patients).

To identify G., the level of serum iron is also examined (if it decreases, iron supplements are prescribed) and the degree of blood saturation with transferrin. A reliable diagnostic criterion is the detection of iron in a liver biopsy, in a bone marrow treponate using the Perls method (see Perls method), as well as counting the number of sideroblasts in a bone marrow aspirate.

Diagnostic value

In areas of hemorrhage, with extravascular destruction of erythrocytes, local hemorrhage occurs. General hemorrhage is observed with various diseases hematopoietic system (anemia, some forms of leukemia), intoxication with hemolytic poisons, some infectious diseases (relapsing fever, brucellosis, malaria, etc.), frequent blood transfusions, etc. Rarely, general hemolysis manifests itself as hereditary disease- hemochromatosis (see). The latter is characterized by the deposition in tissues, in addition to hemosiderin, of another iron-containing pigment - hemofuscin, as well as lipofuscin.

G. is often a complication of liver cirrhosis [Powell, Williams (L. W. Powell, R. Williams)], diabetes. During experimental removal of the pancreas in animals, generalized hemosiderosis is observed. Hemosiderosis is found in patients with hemoglobinopathies (see), iron-refractory anemia (see).

Treatment

Treatment measures are carried out primarily in relation to the underlying disease. In addition, bloodletting is used, which is especially effective for diffuse G. accompanying idiopathic hemachromatosis. Bloodletting in a volume of 500 ml is equivalent to the removal of 200 mg of iron. However, in case of iron-refractory anemia, this method, which requires constant and systematic blood transfusions, is not justified [R. M. Bannerman]. In the treatment of secondary hepatitis (complications of liver cirrhosis), the drug diethylenetriamine penta-acetate [McDonald, Smith (R. A. McDonald, R. S. Smith)] is effective; however, its injections are painful and sometimes cause side effects. The introduction of desferal into the wedge and practice has opened up new opportunities in the treatment of G. of various origins. Desferal is usually administered intramuscularly at a dose of 1-3 g per day. The duration of one course of treatment is at least 3 weeks. There are instructions [F. Wohler] on the use of desferal for a year or more in patients with idiopathic hemochromatosis. The main criterion determining the duration of treatment is the excretion of iron in the urine; if daily iron excretion does not exceed 1.0-1.5 mg, deferoxamine injections are stopped. From other to lay down. agents, you can specify complexing compounds - thetacine-calcium (see) and pentacine (see).

Essential pulmonary hemosiderosis (tsvetn. fig. 26 and 27) occupies a special place, because it is fundamentally different in etiology, pathogenesis and clinic from hemochromatosis. Deposits of hemosiderin are found only in the lungs, which is reflected in its old names - brown induration of the lungs, essential brown induration of the lungs, pulmonary stroke, congenital bleeding into the lungs (see Idiopathic pulmonary hemosiderosis).

Bibliography: Dolgoplosk N. A. and Skaldina A. S. A case of essential hemosiderosis of the lungs, Vestn, rentgenol, i radiol., No. 1, p. 88, 1971, bibliography; Martynov S. M. and Sheremeta N. A. About transfusion hyperhemosideroses and hemochromatosis in leukemia, hemoblastosis and aplastic anemia* in the book: Sovrem. Problem, hematol. and overflow, blood, ed. A. E. Kiseleva et al., c. 38, p. 243, M., 1966; Fainshtein F. E. et al. The use of desferal and some data on hemosiderosis in hypo- and aplastic anemia, Probl.< гематол. и перелив, крови, т. 13, № 8, с. 31, 1968, библиогр.; Хуцишвили Г. Э. Десферал-тест в диагностике гемосидероза у больных гемоглобинопатиями, Лаборат, дело, № 9, с. 660, 1971* библиогр.; Blood and its disorders, ed. by R. M. Hardisty a. D. J. Weatherall, Oxford, 1974; Bothwell Т. H. a. Finch C. A. Iron metabolism, Boston, 1962; Clinical symposium on iron deficiency, ed., by L. Hallberg a. o., L.- N. Y., 1970; Iron metabolism, ed. by F. Gross, B., 1964; Mac Donald R. A. Hemochromatosis and hemosiderosis, Springfield, 1964, bibliogr.; Roberts L. N., Montes-s o r i G. a. P a 11 e r s o n J. G. Idiopathic pulmonary hemosiderosis, Amer. Rev. resp. Dis., v. 106, p. 904, 1972.

L. A. Danilina.

Which accumulates in the blood due to the breakdown of red blood cells, which are blood cells that carry oxygen to tissues and internal organs. Their lifespan is 120 days, after which they disintegrate. Therefore, the destruction of red blood cells occurs in a normal state. But if too many blood cells break down, there is an excess accumulation of the pigment hemosiderin in the blood. Read more about the causes, symptoms, features of diagnosis and treatment of this pathology later in the article.

Causes of pathology

Hemosiderin is a substance that, when accumulated in excess in the body, causes the development of a disease called hemosiderosis. There are two groups of causes of this pathology: exogenous and endogenous. In the first case, there is an impact external factors on the body. In the second case, the disease develops due to a violation of the internal environment of the body.

Endogenous factors that lead to increased hemosiderin deposition include:

acute inflammatory diseases of infectious origin - malaria, brucellosis;
toxic poisoning;
the effect of certain medications;
excessive intake of iron into the body with medications containing it (Sorbifer, Maltofer);
blood transfusion with an incompatible group or Rh factor.

Among them, the greatest attention is paid to heredity. There are some genetic diseases, in which excessive deposition of hemosiderin occurs in the brain, liver and other internal organs. These are, first of all, the following pathologies:

thalassemia - a disorder of the synthesis of one of the hemoglobin chains;
sickle cell anemia is a congenital disorder of the shape of red blood cells;
enzymopathies - a group of diseases in which there is a lack of any enzyme for the formation of hemoglobin;
Membranopathies are congenital disorders of the structure of red blood cells.

Separately allocate autoimmune diseases, as the cause of the development of hemosiderosis.

Forms of the disease

Hemosiderin is a substance that can accumulate both throughout the body, in almost all internal organs, and in isolation, that is, in a specific place. In the first case, they talk about a generalized, or general form of the disease. In the second case, local, or local, hemosiderosis develops.

The occurrence of general hemosiderosis occurs against the background of any systemic pathology. Then hemosiderin accumulates in the brain, liver and other organs. At local form the pigment collects in localized areas of the human body. For example, in the cavity of a tubular organ or in a hematoma.

Depending on the cause of development, two more groups of the disease are distinguished:

primary - the reasons for this form have not yet been clarified;
secondary - develops against the background of other diseases.

The following pathological conditions can be the main causes of secondary hemosiderosis:

leukemia - malignant bone marrow lesion;
cirrhosis of the liver;
infectious diseases;
skin diseases: pyoderma, eczema, dermatitis;
hypertonic disease with severe course;
frequent blood transfusions;
hemolytic anemia.

In fact, there are many more reasons for the development of secondary hemosiderosis, so only the main ones are presented in the previous section and above.

Risk factors for developing the disease

Separately, factors are identified that do not directly lead to increased hemosiderin deposition, but increase the risk of this pathological condition. These include:

constant hypothermia of the body;
chronic stress;
excessive physical exercise;
uncontrolled use of diuretics, paracetamol, and some antibiotics.

What organs are affected by hemosiderosis?

Hemosiderin is a pigment that can accumulate in almost any internal organ. But the most common defeat is:

liver;
kidney;
spleen;
skin;
bone marrow;
salivary or sweat glands;
brain.

Skin hemosiderosis: manifestations

The most striking manifestations are the accumulation of hemosiderin in the skin. In almost all patients, the main symptom is the formation of dark brown spots on the legs. Typically, areas of pigmentation have a large diameter, but sometimes small, almost pinpoint rashes occur. In some patients, this occurs due to damage to the capillaries of the skin.

The shade of the rash can vary: from the color of red brick to dark brown or yellow. In addition to spots, other elements of the rash appear: nodules, papules, plaques. The patient is concerned about itching of the affected areas of the skin.

Hemosiderosis of the liver: symptoms

The deposition of hemosiderin in the liver tissue is manifested, first of all, by an increase in the size of the organ. This leads to stretching of the capsule surrounding the liver. The patient feels it as a dull pain on the right under the rib. With a significant increase, asymmetry of the abdomen occurs and it bulges on the right. Palpation of the abdomen in these sections is also painful.

With a long-term process, liver function is gradually impaired. This is manifested by an enlarged abdomen due to the accumulation of fluid in it, varicose veins veins of the stomach and esophagus, hemorrhoidal veins, yellowing of the skin and sclera, hemorrhagic rashes.

Kidney hemosiderosis: symptoms

The accumulation of pigment in the kidneys not only leads to changes in the urine, but also to certain clinical manifestations. Hemosiderin affects the renal tubules and glomeruli, which leads to disruption of blood filtration and the release of protein and carbohydrates from it. As a result, hypoproteinemia develops - a decrease in protein concentration in the blood.

The patient complains of the appearance of edema. They first appear on the face, and in advanced cases cover the entire body. The patient is concerned about general weakness and fatigue.

Long-term damage to kidney function leads to dysfunction of other organs and systems.

Brain damage

Hemosiderin deposition in the brain has highly variable clinical manifestations. It all depends on which department the lesion is localized in.

Hemosiderin accumulation causes death nerve cells, destruction of the myelin sheath of nerves. Often, patients with cerebral hemosiderosis have previous hemorrhage into the parenchyma, removal of tumors, and hemorrhagic strokes.

Typical clinical manifestations of hemosiderin accumulation in the brain are:

imbalance - ataxia;
hearing impairment such as sensorineural hearing loss;
mental disorders;
dysarthria - speech disorder;
movement disorders.

Diagnostics

Diagnosis of hemosiderosis must be comprehensive. Coordinated work of doctors of different specializations is often necessary: dermatologist, neurologist, pulmonologist, infectious disease specialist and others. It all depends on which organ is predominantly affected.

The diagnostic search begins with a detailed questioning of the patient about his complaints, their dynamic development, and the presence of previous diseases. Only after this are additional examination methods prescribed.

Regardless of the form of the disease, the following diagnostic methods are prescribed:

Complete blood count - a reduced number of red blood cells and hemoglobin is determined.
Determination of the presence of hemosiderin in urine.
The level of iron in the blood serum is determined.
Analysis of the binding capacity of iron in the body.
Biopsy of the affected tissue with histological examination to detect hemosiderin deposits.

Only histological examination A biopsy allows one to make a diagnosis of hemosiderosis with one hundred percent certainty. When examining a piece of tissue under a microscope, macrophages with hemosiderin are found, since it is these cells that are the first to “eat” the excess pigment.

Also, depending on which organ the doctor suspects is damaged, he prescribes the following examination methods:

magnetic resonance imaging of the brain;
computed tomogram;
ultrasonography;
radiography;
bronchoscopy.

Another effective one laboratory method diagnostics - desferal test. To carry it out, the patient is administered 500 mg of desferal. A minimum of 6 hours and a maximum of 24 hours after the injection of the drug, the patient’s urine is collected and the amount of iron in it is examined.

Treatment of the disease

Since on modern stage Most attention is paid to the autoimmune course of hemosiderosis; drugs from the group of corticosteroids are considered priority drugs. They oppress immune system, thereby reducing the production of antibodies against one’s own red blood cells. These drugs include Dexamethasone and Prednisolone. But glucocorticoids help only 40-50% of cases. In the absence of their effectiveness, the patient is prescribed cytostatics (Methotrexate, Azathioprine).

Drugs are also prescribed that improve tissue trophism, cell metabolism, and increase the supply of oxygen to them. Such drugs include:

Venotonics. They increase the elasticity of vascular walls, improve blood flow in the brain tissues - “Detralex”, “Doppelhertz”.
B vitamins. Improve conductivity nerve impulse, condition of the rope fabric.
Vitamin C. Increases strength vascular wall.
Angioprotectors. They have a vitamin C-like effect - “Etamzilat”, “Vincamine”.
Nootropics. Improve blood flow in the brain and accelerate cell metabolism - Cerebrolysin, Phenibut.
Neuroleptics. Prescribed symptomatically only if there is mental disorders the patient has Aminazine.

In case of hemosiderosis of the kidneys and significant impairment of their function, plasmapheresis or hemodialysis is prescribed.

Thus, excessive accumulation of hemosiderin is a serious pathological condition. It requires the earliest possible diagnosis and timely treatment, since in advanced cases hemosiderosis leads to severe dysfunction of internal organs. Often these disorders are irreversible.

General hemosiderosis is always a secondary pathology, the development of which is caused by autoimmune processes, intoxications, infections or blood diseases. The most common causes of hemosiderosis are:

hemolytic anemia;
poisoning with hemolytic poison (quinine, lead, sulfonamides);
leukemia;
multiple blood transfusions;
cirrhosis of the liver;
Rhesus conflict;
infectious diseases (relapsing fever, malaria, brucellosis, sepsis);
decompensated heart failure;
chronic venous insufficiency;
diabetes;
dermatitis;
eczema.

Some experts believe that hereditary predisposition and immune system disorders play an important role in the pathological mechanism of hemosiderosis development.

Forms of the disease

According to the degree of spread of the pathological process, the following are distinguished:

general (generalized) hemosiderosis - it is caused by intravascular (endovascular) hemolysis of red blood cells that occurs against the background of any systemic processes. Hemosiderin is deposited in the spleen, red bone marrow, and liver, as a result of which they acquire a rusty, brown color;
local (local) hemosiderosis - develops as a result of extravascular (extravascular) hemolysis. The accumulation of hemosiderin can be observed both in small areas of the body (in the area of hematomas) and within an organ (for example, the lung).

According to the characteristics of the clinical course, the following are distinguished:

hemosiderosis of the skin (ocher dermatitis, purpuric angiodermatitis, lichenoid pigmentary dermatitis, senile hemosiderosis, annular telangiectatic purpura of Majocchi, Schamberg's disease);
pulmonary idiopathic hemosiderosis (brown induration of the lungs).

Pulmonary hemosiderosis progresses and leads to disability in patients. It can be complicated by massive pulmonary hemorrhages, leading to rapid death.

In some cases, hemosiderin deposition causes dysfunction of the affected organ and the development of structural changes in it. This form of hemosiderosis is called hemochromatosis.

Symptoms

The clinical picture of hemosiderosis depends on the form of the disease.

Pulmonary idiopathic hemosiderosis is diagnosed mainly in children and young people and is characterized by periodically occurring pulmonary hemorrhages of varying severity, increasing respiratory failure and persistent hypochromic anemia.

In the acute phase of the disease the following are observed:

dyspnea;
chest pain;
cough with bloody sputum;
pale skin;
icterus sclera;
cyanosis of the nasolabial triangle;
weakness;
dizziness;
increase in body temperature.

During the period of remission, the clinical manifestations of the disease are very weak or completely absent. After each period of exacerbation of idiopathic pulmonary hemosiderosis, the duration of the remission period decreases.

The cutaneous form of hemosiderosis occurs more often in men over 40 years of age. The course is chronic and benign. Brick-red pigment spots appear on the skin of the forearms, hands, legs and ankles. Over time, the color of the rash elements changes to yellowish, dark brown or brown. Damage to internal organs does not occur in the cutaneous form of hemosiderosis, and the general condition does not suffer.

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Diagnostics

Diagnosis of hemosiderosis is based on the characteristic clinical signs of the disease, data from laboratory and instrumental examination methods. A general blood test is prescribed and serum iron concentration is determined.

To confirm the diagnosis, a puncture biopsy of the bone marrow, lung, liver or skin is performed, followed by histological examination of the resulting tissue.

General hemosiderosis is always a secondary pathology, the development of which is caused by autoimmune processes, intoxications, infections or blood diseases.

For hemosiderosis of the lungs, the following is also carried out:

spirometry;
microscopic examination of sputum;
lung perfusion scintigraphy;
chest x-ray;
bronchoscopy.

Treatment

Therapy for pulmonary hemosiderosis begins with the administration of corticosteroids. If it is ineffective, patients are prescribed immunosuppressants and undergo plasmapheresis sessions. In some cases, it is possible to achieve stable remission after removal of the spleen (splenectomy). In addition, symptomatic therapy is carried out with the prescription of bronchodilators, hemostatics and other drugs (depending on the manifestations of the disease).

Treatment of cutaneous hemosiderosis consists of local application of corticosteroid ointments. Angioprotectors, calcium preparations, rutin, and ascorbic acid are also prescribed. In severe cases of the disease, the use of deferoxamine and PUVA therapy is indicated.

Possible complications and consequences

The most common complications of pulmonary hemosiderosis:

recurrent pneumothorax;
pulmonary hemorrhage;
pulmonary infarction;
expansion and hypertrophy of the right heart;
pulmonary hypertension;
chronic respiratory failure.

Forecast

The prognosis for pulmonary hemosiderosis is serious. The disease progresses and leads to disability in patients. It can be complicated by massive pulmonary hemorrhages, leading to rapid death.

With the cutaneous form of hemosiderosis, the prognosis is favorable.

Pulmonary idiopathic hemosiderosis is diagnosed mainly in children and young people.

Prevention

Prevention of disturbances in hemosiderin metabolism involves timely treatment of hematological, vascular and infectious diseases, prevention of chemical or drug intoxication, and blood transfusion complications.

Etiology

Since such a disease in children and adults can be primary or secondary, the predisposing factors will differ.

In the first case, the pathogenesis and etiology of hemosiderosis remain not fully understood, however, clinicians put forward several assumptions, including:

genetic predisposition;
immunopathological nature of the pathology;
congenital disorders metabolic processes.

As for secondary hemosiderosis, as possible reasons may act:

hemolytic anemia;
leukemia;
liver damage by cirrhosis;
the course of some diseases of an infectious nature - this includes sepsis and relapsing fever, malaria and brucellosis;
frequent blood transfusions;
poisoning chemicals and poisons. The most common provocateurs are sulfonamides, lead and quinine;
the course of diabetes mellitus and other metabolic disorders;
malignant arterial hypertension;
venous insufficiency in chronic form;
dermatitis and neurodermatitis;
eczema and pyoderma;
any integrity violations skin.

The manifestation of the disease can be provoked by the following reasons:

prolonged hypothermia of the body;
physical fatigue;
stress and nervous tension;
uncontrolled reception of some groups medicines, in particular diuretics, non-steroidal anti-inflammatory drugs, as well as paracetamol and ampicillin.

Not the last place in the formation of such a pathological process is occupied by:

excess iron in the body - this can be achieved by consuming foods or medications that contain large amounts of this substance;
blood transfusion procedure, especially if it is performed several times a week. In such cases, they talk about the occurrence of transfusion hemosiderosis;
increased absorption of hemosiderin by the digestive system;
violation of hemosiderin metabolism;
hypothermia of the body.

Classification

According to the degree of prevalence of the pathological process, there are:

generalized or general hemosiderosis- is formed against the background of some system process. In such situations, hemosiderin accumulates in the spleen and liver, as well as in the red bone marrow, causing them to change color and become rusty or brown;
local, focal or local hemosiderosis- differs in that the accumulation of iron-containing intracellular pigment can occur both in small areas of the body, for example, in the area of hematomas, and in the cavity of one of the internal organs. This is most often observed in lung tissue.

According to the etiological factor, hemosiderosis is:

primary - the reasons remain unknown today;
secondary - develops against the background of any of the above ailments.

Independent types of the disease include:

hemosiderosis of the skin;
idiopathic pulmonary hemosiderosis, which in the medical field is called brown purpura of the lungs.

In cases of damage to the skin, the disease can occur in several variations, which is why it is divided into:

Schamberg's disease;
ring-shaped telangiectatic purpura of Majocchi;
senile hemosiderosis;
lichenoid pigmentosa or angiodermitis purpurosa;
ocher dermatitis.

The secondary form of pathology also has its own classification:

liver hemosiderosis;
kidney hemosiderosis;
bone marrow hemosiderosis;
hemosiderosis of the spleen;
hemosiderosis with damage to the salivary or sweat glands.

Symptoms

The clinical picture of such a disease in children and adults is directly dictated by the type of its course.

Thus, idiopathic pulmonary hemosiderosis is represented by the following manifestations:

productive cough, i.e. with sputum production;
hemoptysis;
shortness of breath;
increase in temperature values;
pain syndrome localized in the sternum;
pale skin;
icteric sclera;
cyanosis of the nasolabial triangle;
severe dizziness;
weakness and decreased performance;
lack of appetite;
increased heart rate;
decreased blood pressure;
simultaneous change in the volumes of the liver and spleen in a larger direction.

Given that brown induration of the lungs is a serious illness, such a diagnosis is made quite rarely during a person’s lifetime.

Hemosiderosis of the skin is primarily expressed in the appearance of brown pigment spots and in the vast majority of cases is localized on the lower part of the legs. In addition, the symptoms of this variation of the disease also include:

the appearance of a hemorrhagic rash or small pigment spots that vary in diameter from one millimeter to 3 centimeters;
involvement in the pathology of the legs, forearms and hands;
variability in the shade of the rashes - they can be brick-red, brown, dark brown or yellowish;
slight skin itching;
the formation of petechiae, nodules, lichenoid papules and plaques in problem areas of the skin.

Hemosiderosis of the liver is expressed in:

an increase in the size and density of the affected organ;
pain upon palpation of the projection of this organ;
accumulation of a large amount of fluid in the peritoneum;
increased blood tone;
yellowness of the skin, visible mucous membranes and sclera;
pigmentation upper limbs, face and armpits.

In cases where the kidneys are involved in the pathological process, the presence of:

swelling of the lower extremities and eyelids;
food aversions;
cloudy urine;
pain in the lumbar region;
general weakness and weakness;
dyspepsia.

It is advisable to relate all of the above symptoms to children and adults.

Diagnostics

Since hemosiderosis is primarily characterized by changes in blood composition, it is quite natural that laboratory tests will form the basis of diagnosis.

If characteristic symptoms occur, you should seek help from a therapist who will carry out initial diagnostic procedures and prescribe general examinations, and if necessary, refer the patient for additional consultation to other specialists.

The first step in establishing the correct diagnosis is aimed at:

studying the medical history - to search for pathological factors leading to secondary hemosiderosis;
familiarization with a person’s life history - to establish possible sources of the primary form of the disease;
a thorough physical examination of the patient, which must include examination of the condition of the skin, palpation of the anterior wall abdominal cavity, pulse and blood pressure measurement;
detailed survey of the patient - to compile a complete symptomatic picture course of the disease.

Laboratory studies are based on:

in general clinical analysis blood;
blood biochemistry;
PCR tests;
general urine analysis;
disferal test;
microscopic examination of sputum.

The most valuable in terms of diagnostics are the following instrumental procedures:

biopsy of the affected segment, i.e. skin, liver, kidneys, lung, bone marrow, etc.;
spirometry;
bronchoscopy;
scintigraphy;
X-ray of the sternum;
CT and MRI.

Treatment

This disease can be cured using conservative therapy methods used in any variant of its course.

Treatment of hemosiderosis includes:

taking glucocorticoids;
use of immunosuppressants and iron supplements;
performing plasmapheresis;
use of medications to relieve symptoms;
oxygen inhalation;
cryotherapy;
vitamin therapy;
taking medications containing calcium and angioprotectors;
PUVA therapy - for cutaneous hemosiderosis.

Surgical intervention is not used in the treatment of the disease.

Possible complications

Idiopathic pulmonary hemosiderosis and other types of the disease, in the absence of therapy, are fraught with the following consequences:

pulmonary infarction;
pulmonary hypertension;
internal hemorrhages;
recurrent pneumothorax;
chronic respiratory failure;
pulmonary heart syndrome;
hypochromic anemia.

Kinds

Local hemosiderosis develops as a result of extravascular hemolysis in the lesion - in an organ or hematoma. Excessive pigment accumulation does not cause tissue damage. If there are sclerotic changes in the organ, its function is impaired.

General hemosiderosis is the result of intravascular hemolysis and significant deposition of hemosiderin in the internal organs. Hepatocytes of the liver, cells of the spleen and other internal organs are predominantly affected. Excess pigment makes them brown or “rusty.” This occurs in systemic diseases.

The following nosological forms are distinguished:

Essential pulmonary hemosiderosis,
Hereditary hemochromatosis,
Cutaneous hemosiderosis,
Hemosiderosis of the liver,
Idiopathic hemosiderosis.

In turn, cutaneous hemosiderosis is divided into the following forms: Schamberg disease, Majocchi disease, Gougerot-Blum disease, ocher dermatitis.

Causes

The etiology of the disease is not fully understood. Hemosiderosis is a secondary condition caused by pathological processes existing in the body.

The disease develops in individuals who have:

Hematological problems - hemolytic anemia, leukemia,
Infectious diseases - sepsis, brucellosis, typhoid, malaria,
Autoimmune processes and immunopathologies,
Endocrinopathies - diabetes mellitus, hypothyroidism,
Congenital defects and anomalies of the vascular wall,
Vascular pathologies - chronic venous insufficiency, hypertension,
Heart failure in the stage of decompensation or cirrhosis of the liver,
Intoxication syndrome,
Rhesus conflict.

Of great importance in the development of pathology are frequent blood transfusions, hereditary predisposition, skin diseases, abrasions and wounds, hypothermia, taking certain medicines, excessive consumption of iron in food.

Symptoms

The clinical picture of hemosiderosis is determined by the location of the lesion. The disease begins suddenly and develops gradually.

In the skin form, the rash lasts for months and years and is accompanied by itching of varying intensity. The pigment spots are clearly defined, have a reddish color and remain on the skin even with pressure.

Pulmonary hemosiderosis is characterized by the appearance of shortness of breath at rest, anemia, wet cough with bloody discharge, a rise in temperature to febrile levels, increasing signs of respiratory dysfunction, hepatosplenomegaly. Exacerbations after a few days are replaced by remission, in which the hemoglobin level normalizes, and Clinical signs gradually disappear.

Pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis is a severe chronic pathology, the main pathomorphological components of which are: repeated hemorrhages in the alveoli, breakdown of red blood cells and a significant accumulation of hemosiderin in the pulmonary parenchyma. As a result, patients develop persistent pulmonary dysfunction.

Symptoms acute form diseases are:

Productive cough and hemoptysis,
Pale skin
Scleral injection,
brokenness,
Dyspnea,
Chest pain,
Arthralgia,
Temperature rise
Cardiopalmus,
Drop in blood pressure
Hepatosplenomegaly.

Remission is characterized by the absence of complaints and severe clinical symptoms. During this period, patients remain able to work. Over time, exacerbations of pathology occur more often, and remission becomes shorter.

IN severe cases the chronic course of hemosiderosis is manifested by symptoms pulmonary heart, pneumonia, pneumothorax and can be fatal.

Brown induration of the lungs is a serious disease that is rarely diagnosed during life. Autoantibodies are formed in the blood of patients in response to exposure to a sensitizing antigen. An antigen-antibody complex is formed, and autoallergic inflammation develops, for which the lungs become the target organs. Pulmonary capillaries expand, red blood cells penetrate from the vascular bed into the lung tissue, and hemosiderin begins to be deposited in it.

Skin hemosiderosis

Skin hemosiderosis - dermatological disease, in which pigment spots and various rashes appear on the skin of patients. The appearance of areas of pigmentation and petechiae is caused by the accumulation of hemosiderin in the dermis and damage to the capillaries of the papillary layer.

The spots on the skin vary in color and size. Fresh rashes are usually bright red, while older ones are brownish, brown or yellow. Spots up to three centimeters in size are localized on the lower extremities, hands and forearms. Petechiae, nodules, papules, and plaques often appear on the affected skin. Patients complain of slight burning and itching.

In the patient's dermis, the structure of the endothelium of the capillaries changes, and the hydrostatic pressure in them increases. Plasma leaves the vascular bed, and red blood cells are excreted along with it. The capillaries dilate, clumps of hemosiderin are deposited in the endothelium, histocytes and endothelial cells are affected, and perivascular infiltration develops. This is how the process of hemosiderin deposition occurs in the skin. In patients, clinical blood tests usually reveal thrombocytopenia and impaired iron metabolism.

Hemosiderosis of the skin can occur in various clinical forms, among which the most common are: Majocchi disease, Gougerot-Blum disease, orthostatic, eczematid-like and itchy purpura.

Schamberg's disease

Deserves special attention Schamberg's disease. This is quite common autoimmune pathology chronic course, characterized by the appearance of red dots on the skin, similar to a mark from a regular injection. Circulating immune complexes are deposited in the vascular wall, autoimmune inflammation of the endothelium develops, and intradermal petechial hemorrhages appear. Hemosiderin in large quantities accumulates in the papillary layer of the dermis, which is clinically manifested by the appearance of symmetrical brown spots on the skin. They come together and form plaques or entire areas that are yellow or brown. Bright red rashes appear along the edges of such plaques. In patients, petechiae and hemorrhages the size of a pea are also present on the skin, which merge with each other and form large lesions. Over time, the plaques in the center atrophy. The general health of the patients remains satisfactory. The prognosis of the pathology is favorable.

Cutaneous hemosiderosis, unlike pulmonary and general hemosiderosis, can be easily corrected. Patients feel well and recover quickly.

Hemosiderosis of internal organs

Systemic or generalized hemosiderosis develops in the presence of massive intravascular hemolysis of red blood cells. The disease is characterized by damage to internal organs and is severe clinical course. Patients with general hemosiderosis complain of bad feeling, their skin color changes, and frequent bleeding occurs.

Hemosiderosis of the liver It can be idiopathic or primary, as well as secondary. Hemosiderin is deposited in hepatocytes. The liver enlarges and thickens, becomes painful on palpation, ascites, hypertension, jaundice of the skin and sclera, splenomegaly, pigmentation of the hands, face and armpits occur. IN terminal stage if left untreated, acidosis develops. The patient falls into a coma.
Kidney hemosiderosis characterized by the appearance of brown granules in the parenchyma of the organ. Patients develop clinical signs of nephrosis or nephritis. Protein is found in the urine, and increased lipid content is detected in the blood. The main symptoms of kidney hyposiderosis are: swelling of the feet, legs and eyelids, weakness, lack of appetite, cloudiness in the urine, lower back pain, dyspepsia. If treatment is not started on time, serious complications will arise - renal failure, which often ends in the death of patients.
Hemosiderin can be deposited in the brain, spleen and other organs, accumulate in macrophages and vascular endothelium. This leads to the development dangerous diseases, severe complications and even death.

Systemic hemosiderosis poses a danger to the life of patients, making it miserable and short. Damage to internal organs often ends in the development of serious consequences.

Diagnostics

After listening to patient complaints, collecting anamnesis and conducting a general examination, specialists move on to laboratory and instrumental methods research.

In a clinical laboratory, blood is taken for a general analysis to determine serum iron and total iron-binding capacity.
Biopsy of the affected tissue and histological examination of the biopsy sample has great importance to make a diagnosis.
The desferal test allows you to determine hemosiderin in the urine after intramuscular injection"Desferala."
Dermatoscopy of the rash area - microscopic examination of the papillary layer of the dermis.

Among additional diagnostic methods, the most common are next research lungs:

radiographic,
tomographic,
scintigraphic,
bronchoscopic,
spirometric,
microscopic and bacteriological examination sputum.

Treatment

Treatment of hemosiderosis begins with the implementation of general medical recommendations:

Eat right, eliminate food allergens from your diet, spicy and fried foods, smoked meats, alcohol;
Avoid injuries, hypothermia, overheating and overexertion;
Timely identify and sanitize foci of chronic infection in the body;
Treat concomitant diseases;
Do not use cosmetics which can cause allergies;
Fight bad habits.

Drug therapy consists of prescribing medications to patients:

Local and systemic corticosteroids - Prednisolone, Betamethasone, Dexamethasone and drugs based on them,
Anti-inflammatory drugs - Ibuprofen, Indomethacin,
Disaggregants - “Aspirin”, “Cardiomagnyl”, “Acetylsalicylic acid”,
Immunosuppressants - “Azathioprine”, “Cyclophosphamide”,
Angioprotectors - “Diosmina”, “Hesperidin”,
Antihistamines - “Diazolina”, “Suprastina”, “Tavegila”,
Nootropic drugs - “Piracetam”, “Vinpocetine”, “Maxidol”,
Multivitamins and polyminerals - ascorbic acid, routine, calcium supplements.

Symptomatic correction includes long-term use of iron supplements, hemostatic agents, multivitamins, bronchodilators, and oxygen therapy. Patients undergo extracorporeal hemocorrection: hemosorption, plasmaphoresis, cryoprecipitation, blood therapy. In severe cases, splenectomy and blood transfusions give good results.

Facilities traditional medicine, used to strengthen the vascular wall and reduce the manifestations of hemosiderosis: infusion of mountain arnica and hazel bark, decoction of thick-leaved bergenia.

Prevention

Hemosiderosis - chronic illness, characterized by alternating exacerbation and remission. After proper treatment and stabilization of the patient’s condition, it is necessary to observe preventive actions, preventing a new exacerbation. These include Spa treatment, proper nutrition And healthy image life.

Preventive measures to prevent the development of hemosiderosis:

Timely and adequate treatment of acute infectious and dermatological pathologies,
Solving problems with blood vessels,
Control of body weight, cholesterol and blood pressure,
Excluding poisoning and intoxication.