Hemosiderosis: symptoms and treatment. Is Schamberg's disease a cosmetic defect or a serious disease? Deposition of hemosiderin in the skin

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 supply of iron under normal conditions 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 of 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.

Food products contain iron in quantities many times higher than required. 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 need for iron in the body is small, 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 (blue) in the epithelium of convoluted tubules of the kidneys in 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; Using this test, you can exclude iron deficiency anemia (a common misdiagnosis in patients with thalassemia).

To identify G., the level is also examined serum iron(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 red blood cells, local hemorrhage occurs. General hematopoiesis is observed in various diseases of the hematopoietic system (anemia, some forms of leukemia), intoxication with hemolytic poisons, some infectious diseases (relapsing fever, brucellosis, malaria, etc.), and frequent blood transfusions. etc. Rarely, general G. manifests itself as a 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.

Hemosiderosis is a metabolic disorder caused by an excess content of the pigment hemosiderin in the body's cells. Hemosiderin is formed as a result of the biochemical breakdown of hemoglobin under the influence of endogenous enzymes. This substance is involved in the transport and storage of certain chemicals and compounds. Accelerated breakdown of red blood cells, excessive absorption of hemosiderin in the intestine, disruption of metabolic processes in the body lead to excessive formation of pigment and the development of hemosiderosis.

Hemosiderosis has several equivalent names: pigmentary hemorrhagic dermatosis, chronic pigmentary purpura, capillaritis. This dystrophic pathology can occur in various forms:

Local or local, associated with damage to the skin and lungs,
General or generalized, in which hemosiderin accumulates in the liver, spleen, kidneys, bone marrow, salivary and sweat glands.

Various forms of pathology are manifested by similar clinical signs: a red or brown hemorrhagic rash, hemoptysis, anemia, and general asthenia of the body. The disease most often develops in men in adulthood. In children, the pathology is extremely rare.

Hemosiderosis is a blood and metabolic disease that is difficult to treat. This is not just a cosmetic defect, but serious problem, causing dysfunction of internal organs and systems.

Diagnosis and treatment of pathology is carried out by doctors of various specialties: hematologists, dermatologists, pulmonologists, immunologists. Patients are prescribed glucocorticosteroids, cytostatic and angioprotective drugs, multivitamins, plasmapheresis.

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.

hemosiderin deposits in tissues (idiopathic pulmonary hemosiderosis)

General hemosiderosis - result intravascular hemolysis and significant deposition of hemosiderin in 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 –,
Infectious diseases - sepsis, brucellosis, typhoid, malaria,
Autoimmune processes and immunopathologies,
Endocrinopathies - diabetes, hypothyroidism,
Birth defects and anomalies vascular wall,
Vascular pathologies – hypertension,
in the stage of decompensation or cirrhosis of the liver,
Intoxication syndrome,

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, a wet cough with bloody discharge, a rise in temperature to febrile values, an increase in signs of respiratory dysfunction, and 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 of the acute form of the disease 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 pronounced 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 of pneumonia, pneumothorax and may end in death.

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

Hemosiderosis of the skin is a dermatological disease in which age spots and various rashes appear on the skin of patients. The appearance of areas of pigmentation is due to the accumulation of hemosiderin in the dermis and damage to the capillaries of the papillary layer.

cutaneous hemosiderosis

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 in clinical analysis blood usually reveals 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 a fairly common chronic autoimmune pathology, characterized by the appearance of red dots on the skin, similar to the mark of a regular injection. Circulating immune complexes are deposited in the vascular wall, autoimmune inflammation of the endothelium develops, and intradermal petechial hemorrhages appear. Hemosiderin accumulates in large quantities in the papillary layer of the dermis, which is clinically manifested by the appearance of symmetrical brown spots. 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.

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, and the total iron-binding capacity is determined.
A biopsy of the affected tissue and histological examination of the biopsy is of great importance for making 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 of sputum.

Treatment

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

Eat right, exclude from diet food allergens, spicy and fried foods, smoked foods, 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 that may cause allergies;
Fight bad habits.

Drug therapy consists of prescribing to patients medicines:

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 competent treatment and stabilization of the patient’s condition, it is necessary to observe preventive measures to prevent 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.

Hemosiderin is an iron-containing pigment that has a yellowish color and is a produced polymer of ferritin. It is deposited as a result of the breakdown of red blood cells, as well as when their metabolic process is disrupted. If there is excessive deposition of such pigment, then the patient is diagnosed with a disease such as hemosiderosis.

A disease such as hemosiderosis mainly develops as a result of other diseases that appeared earlier, but at the same time it can also be independent in nature. Basically, the disease occurs as a result of the following reasons:

Disruption of the process of destruction of red blood cells, as well as an increased amount of iron absorbed by the body.
As a result of blood transfusion.

It is no secret that for the normal functioning of the body, it requires a certain amount of a microelement such as iron every day. Usually, daily norm is 15 mg. If, for even a short period of time, the body receives a large amount of this element, this threatens with an excessive amount of iron-containing pigment accumulated in the tissues, which in turn leads to the development various diseases.

There are two main forms of development of such a disease as hemosiderosis:

General hemosiderosis. Excess hemosiderin in general hemosiderosis is found in the liver, spleen, bone marrow, and other internal organs. It is also worth noting that due to the absence of the property of destroying parenchymal cells, the functions of the organs in which hemosiderin has accumulated are not impaired.

Local hemosiderosis. Occurs as a result of intravascular destruction of red blood cells. When hemoglobin disappears from red blood cells, they turn into pale bodies. After this, the released hemoglobin, as well as part of the red blood cells, are covered by other cells and are generated into hemosiderin. Deposition of hemosiderin in the brain, as well as other organs, is often observed. The disease hemosiderosis can occur both in a specific area of tissue and in the whole organ.

How does excess hemosiderin accumulation manifest itself?

The clinical picture primarily depends on which organ has an excess amount of accumulated iron-containing pigment. At the same time, the symptoms may be very similar, or manifest themselves to a similar extent. It is also noteworthy that hemosiderosis can develop differently in everyone, and therefore the symptoms manifest themselves individually for each patient. The most common manifestations of this disease include the following:

Cough with bloody discharge. This manifests itself as a result of the accumulation of hemosiderin in the sputum.
Problem breathing.
Change in skin color.
Pain in the heart and other organs.
Change in liver size.

Accumulation of hemosiderin in the brain and other internal organs

A disease such as hemochromatosis occurs as a result of excessive accumulation of hemosiderin in tissues, as well as internal organs of the body. As a result of the fact that iron tends to accumulate in almost any organ, it becomes the cause of the development of various diseases, in particular diabetes mellitus, cirrhosis of the liver, cardiac or renal failure And so on. The clinical picture is mainly accompanied by general malaise, fatigue, joint pain, and heart failure. When hemosiderin accumulates in the body in large quantities, the color of the affected organs begins to change, and they acquire a yellowish or brown tint.

In order to diagnose the disease, it may require the participation of several doctors at once, in particular a dermatologist, pulmonologist and others. Considering the form of the disease, they may be prescribed various tests, which will help specialists establish an accurate diagnosis and prescribe appropriate treatment. For example, if hemosiderin is detected in the urine (normally it is absent), then this may indicate damage to the body from poisons, infections, and the like. Depending on this, the necessary treatment is prescribed.

How to remove hemosiderin if it has accumulated in excess? Basically, experts prescribe glucocorticosteroids to patients, but they are effective only in 40-50% of cases. If such drugs do not bring the desired effect, immunosuppressants and other medications are used. What are macrophages with hemosiderin?

Macrophages are special cells that are designed to capture various bacteria and dead cells and are one of the lines of defense of the immune system, which helps protect the body from various pathogens. They originate in the bone marrow, where a cell such as the monobullast is initially synthesized. Considering the fact that the accumulation of hemosiderin can be observed in various organs, thereby disrupting their functionality, it is not difficult to assume that hemosiderosis causes a disruption in the development of macrophages. In addition, without proper treatment of the pathological accumulation of iron-containing pigment, other serious diseases may occur, which not only negatively affect the patient’s quality of life, but can also lead to death.

For example, macrophages with hemosiderin in the mammary gland indicate that a cavity has formed in the breast, which is filled with blood inside. This indicates benign dysplasia.

Hemosiderosis can also cause the development of various gastrointestinal diseases, in particular lead to erosion of the antrum of the stomach covered with hemosiderin. Despite the fact that such a disease can be independent in nature, very often it occurs precisely as a result of excessive accumulation of iron-containing pigment, which occurs as a result of a disruption in the absorption of iron by the body.

Hemosiderosis - mixed pigmentary dystrophy, characterized by intra- or extracellular deposition of hemoglobinogenic pigment - hemosiderin.

Classification.

There are two forms of hemosiderosis:

common,
local.

Occurrence.

It occurs very often in tissues as a result of hemorrhages, less often as a manifestation of intravascular hemolysis.

Conditions of occurrence.

General hemosiderosis occurs in malaria, blood transfusion conflicts, autoimmune hemolytic anemia, and poisoning with certain poisons. Local - mainly for ruptured blood vessels.

Mechanisms of occurrence.

General hemosiderosis occurs due to intravascular destruction of red blood cells. The released hemoglobin is captured from the blood by various cells - the so-called sideroblasts, in which it is converted first into ferritin and then into hemosiderin. The role of sideroblasts in this case is played by macrophages of the liver, spleen, bone marrow, as well as hepatopites, epithelial cells of the convoluted tubules of the kidneys, sweat and salivary glands. The convoluted tubule epithelium reabsorbs hemoglobin from primary urine and synthesizes hemosiderin.

Local hemosiderosis occurs as a result of the evolution of hemorrhages in tissues. Fragments of collapsing red blood cells are captured by tissue macrophages - histiocytes - and processed into hemosiderin. The death of these tissue macrophages leads to extracellular deposition of hemosiderin clumps in the tissue.

Macroscopic picture.

With widespread hemosiderosis resulting from hemolysis, the liver, spleen, and especially the bone marrow have a rusty tint. If episodes of hemolysis do not recur, then as the cells in the organs naturally change, their color normalizes.

With local hemosiderosis, the area of former hemorrhage looks like a rust-colored focus or a cavity with walls of the same color.

Microscopic picture.

In widespread hemosiderosis, pigment in the form of golden-brown lumps is detected in the cytoplasm of stellate reticuloendotheliocytes (Kupffer cells) of the liver, in macrophages of the spleen and bone marrow, as well as in epithelial cells - hepatocytes, alveolocytes, epithelial cells of the sweat and salivary glands.

With local hemosiderosis in the area of former hemorrhage, the pigment is detected in the cytoplasm of hepatocytes and in the form of clumps extracellularly. In some tissues, extracellular hemosiderin can persist indefinitely.

To identify hemosiderin, histochemical reactions can be used - the Perls reaction with the formation of Prussian blue and the coloring of pigment clumps in a blue-green color and the Tirman Schmelyder reaction with the formation of Turnboulian blue.

Clinical significance.

With hemosiderosis of the lungs, pigment is deposited along the lymphatic capillaries, which over time leads to stagnation of lymph and compaction lung tissue so-called brown indurania - as a result of which gas exchange in the lungs is disrupted. In other cases, the accumulation of hemosiderin in tissues does not significantly affect their function, but is only a marker of intra- or extravascular hemolysis that has taken place.

Hemosiderosis - main symptoms:

Weakness
Itchy skin
Fever
Dizziness
Loss of appetite
Cardiopalmus
Dyspnea
Enlarged spleen
Chest pain
Liver enlargement
Low blood pressure
Hemoptysis
Pale skin
Cough with phlegm
Decreased performance
Yellowness of mucous membranes
Cloudy urine
Yellowing of the skin
Blue discoloration of the nasolabial triangle
Hemorrhagic rash

Hemosiderosis is a disease that belongs to the category of pigmentary dystrophies, and is also characterized by accumulation in tissues large quantity hemosiderin, which is an iron-containing pigment. The mechanism of development of the disease remains not fully known, however, experts in the field of dermatology have found that the reasons for its formation may differ depending on the form of such a disorder.

Etiology
Classification
Symptoms
Diagnostics
Treatment
Possible complications
Prevention and prognosis

The symptomatic picture is also directly dictated by the type of disease. It is noteworthy that hemosiderosis can affect both internal organs, and the skin. In the second case, patients very rarely complain about deterioration in well-being.

The basis of diagnosis is laboratory blood tests, which mandatory should be supplemented by instrumental procedures and a number of manipulations performed directly by the clinician.

Treatment of hemosiderosis is limited to the use of conservative methods of therapy, in particular taking medications. However, in severe cases of the disease, a procedure such as plasmapheresis may be necessary.

In the international classification of diseases of the tenth revision, this disease is assigned several of its own codes, differing in the course of the disease. Hemosiderosis of the skin has a value of L 95.8. Pulmonary hemosiderosis code according to ICD-10 is J 84, and the idiopathic form of the pathology is J 84.8.

Causes of hemosiderosis

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 metabolic disorders.

As for secondary hemosiderosis, possible causes may include:

hemolytic;
liver damage by cirrhosis;
the course of some diseases of an infectious nature - this includes relapsing fever, malaria, etc.
frequent blood transfusions;
poisoning chemicals and poisons. The most common provocateurs are sulfonamides, lead and quinine;
the occurrence of other metabolic disorders;
malignant;
in chronic form;
eczema and pyoderma;
any violation of the integrity of the skin.

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

prolonged hypothermia of the body;
physical;
stress and nervous tension;
uncontrolled use of certain groups of medications, in particular diuretics, non-steroidal anti-inflammatory drugs, as well as ampicillin.

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

excess 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 of the disease

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

generalized or general hemosiderosis– is formed against the background of some systemic 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:

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 of hemosiderosis

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.

Symptoms of skin hemosiderosis photo.

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 of the 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 perform initial diagnostic procedures and prescribe general examinations, and, if necessary, refer the patient for additional consultation with 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 of the abdominal cavity, measurement of pulse and blood pressure;
a detailed survey of the patient - to compile a complete symptomatic picture of the course of the disease.

Laboratory studies are based on:

general clinical blood test;
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.

Spirometry procedure photo.

Treatment of hemosiderosis

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 calcium-containing 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:

lung;
internal hemorrhages;
recurrent pneumothorax;
chronic respiratory failure;
hypochromic anemia.

Prevention and prognosis

Since in half of the cases it is not possible to find out the cause of the formation of such a disease, preventive recommendations will not be specific, but general. These include:

proper and balanced nutrition;
maintaining a healthy and active lifestyle;
preventing any injuries and hypothermia;
early diagnosis and full treatment pathologies that can provoke the development of secondary hemosiderosis;
exclusion of medicinal and chemical intoxications;
regular passage preventive examination in a medical facility.

Despite the fact that hemosiderosis is quite difficult to diagnose, its prognosis will be relatively favorable - the cutaneous form is best tolerated by people. Very often, other types of the disease lead to disability and the development of complications, which sometimes cause the death of the patient.

What to do?

If you think that you have Hemosiderosis and the symptoms characteristic of this disease, then a general practitioner can help you.

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 internal environment body.

Endogenous factors that lead to increased hemosiderin deposition include:

spicy inflammatory diseases 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.

Autoimmune diseases are separately identified as the cause 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 generalized, or general form diseases. 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. In the 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;
hypertension 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 activity;
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 of the stomach and esophagus, hemorrhoidal veins, yellowing of the skin and sclera, and 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 worried 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 accumulation of hemosiderin in the brain is considered:

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 of a biopsy allows one to make a diagnosis of hemosiderosis with 100% certainty. When examining a piece of tissue under a microscope, macrophages with hemosiderin are found, since these are the 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 at the present 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 the conductivity of nerve impulses and the condition of the vernal tissue.
Vitamin C. Increases the strength of the 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 the patient has mental disorders - "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 maximum early diagnosis and timely treatment, since in advanced cases hemosiderosis leads to severe dysfunction of internal organs. Often these disorders are irreversible.

Hemosiderosis of the skin is a number of dermatological diseases belonging to the group of pigmentary dystrophies. This pathology develops due to an excess amount of iron in the tissues of the body, the level of which is regulated by hemosiderin, an iron-containing pigment. There are local and general hemosiderosis. In the first case - pulmonary and skin, in the second - sweat and salivary glands, bone marrow, kidneys, spleen, liver. To diagnose the disease, it is necessary to determine the level of iron in the body, assess the ability of the blood to bind this element, and determine the amount of hemosiderin in biopsy samples ( biological material) organs and tissues. Treatment depends on clinical picture, includes taking glucocorticosteroids, angiopretectors, cytostatics, vitamins, as well as physiotherapeutic procedures - plasmapheresis, phototherapy (PUVA), etc.

Features of skin hemosiderosis

In most cases, the pathology develops in men. The disease is characterized by brownish-red spots of various shades with clear boundaries. When pressed, they do not change color or disappear, and the inflammatory process does not develop. Depending on the signs and condition of the body, the following forms of skin hemosiderosis are distinguished:

White atrophy. It manifests itself as thinning of the skin on the legs - whitish, slightly sunken, rounded spots with clear boundaries. Gradually, the spots merge, and hyperpigmentation appears around them, caused by telangiectasia and deposition of hemosiderin in the tissues. The process is irreversible, first described in 1929, and most often develops against the background of varicose veins.
Arc-shaped telangiectatic purpura. It appears as yellowish-pink spots 10-15 cm in size, localized on the legs and ankles. Gradually they disintegrate into arcuate elements and semirings, and traces of hemosiderin and telangiectasia appear around them. The pathology was first described in 1934; when diagnosing, it must be differentiated from Majocchi disease.
Eczematic purpura. Numerous eczema-like (erythemo-squamous) yellowish-brown spots appear on the trunk and limbs in combination with a papular-hemorrhagic, sometimes confluent, rash. The rash is in some cases accompanied by itching. At histological examination In addition to hemosiderosis, spongiosis is diagnosed. The disease was first described in 1953.
Itchy purpura. It is similar to eczematid-like purpura, accompanied by severe itching and lichenification - a sharp thickening of the skin, the appearance of a pattern, and pigmentation disorders. The disease was first described in 1954.
Annular telangiectatic purpura. Majocchi's disease is a very rare type of hemorrhagic pigmentary dermatosis.
Lichenoid purpurosa and pigmentary angiodermatitis. On the legs and thighs, reddish-brown lichenoid nodules 3-5 mm in size are formed, less often - small round plaques with a scaly surface. The skin around the affected areas becomes purple and sometimes itchy. The rash may spread to the body, upper limbs, face. The disease is chronic and was first described in 1925.
Orthostatic purpura. Pathological changes skin of the lower extremities, caused by liver and kidney diseases, long stay on your feet, old age, cardiovascular failure. The pathology was first described in 1904.
Ocher dermatitis (Janselm's angiosclerotic purpura, purpuric and pigmented angiodermatitis of the lower extremities, yellow ocher dermatitis). Most often it develops in men who have problems with blood circulation (varicose veins, thrombophlebitis, atherosclerosis, hypertension, etc.), manifests itself on the feet and legs. At first these are tiny hemorrhages, over time - rusty-red lesions 6-10 cm in size. In severe cases - skin atrophy, trophic ulcers. Sometimes - hyperpigmentation and brown-blue spots (plaques) prone to ulceration from 10 to 40 mm in diameter. The disease was first described in 1924.
Progressive pigmentary dermatosis. A rare disease of unknown etiology, it has other names - Schamberg's disease, pigmentary purpura.
Reticular senile hemosiderosis (telangiectatic purpuric dermatitis, senile Bateman's purpura, reticular hemosiderosis). It develops as a result of atherosclerotic changes in the walls of blood vessels caused by natural aging.
Appears as small tan spots, purple petechiae and spider veins(telangiectasia) with localization on back side hands, feet and legs, sometimes the rash is accompanied by itching. The pathology was first described in 1818.

You can see what skin hemosiderosis looks like in the photos below.

Signs of hemosiderosis depend on the form of the disease. Purpura pigmentosa of the skin is more often diagnosed in men over 40 years of age. This is a chronic benign pathology that has the following symptoms:

Pigmented brick red spots.
Localization of the rash on the forearms, hands, legs and ankles.
A gradual change in the color of the spots to brown, dark brown or yellowish.
Temperature increase.

Hemosiderosis of the skin does not spread to the internal organs; during the period of remission, symptoms are mild or absent altogether.

Hemosiderin, which regulates the amount of iron in the body, is formed as a result of the breakdown of hemoglobin under the action of a number of enzymes. It is present in the cells of the liver, spleen, bone marrow, and lymphoid tissue. With overly active synthesis, excess hemosiderin is deposited in skin cells and other organs and tissues. Hemosiderosis of the skin is a secondary disease, the causes of which are:

Autoimmune processes.
Intoxication – poisoning with sulfonamides, lead, quinine, and other hemolytic poisons.
Infections – sepsis, brucellosis, malaria, typhoid.
Blood diseases - multiple blood transfusions, leukemia, diabetes.
Hemolytic anemia.
Rhesus conflict.
Cirrhosis of the liver.
Heart and venous insufficiency.
Eczema, dermatitis.
Immune system disorders.

The results of numerous studies suggest that one of the reasons for the development of skin hemosiderosis is genetic predisposition.

Most often, hemosiderosis of the skin develops in children 3-8 years old; it all begins with anemia and shortness of breath in a calm state. In the acute stage, the temperature rises slightly; if it is a pulmonary form, a cough and sputum with blood appear. During this period, the liver and spleen enlarge, but with treatment the crisis lasts only a few days and the disease goes into remission: the symptoms gradually disappear, the level of hemoglobin in the blood increases.

Diagnosis of skin hemosiderosis

Diagnosis of hemosiderosis requires an assessment of the clinical picture, as well as a number of instrumental and laboratory studies. Usually the doctor prescribes:

Clinical blood test - determines the iron content in the serum.
Desferal test - deferoxamine is administered (intramuscularly), the concentration of iron in the urine is assessed.
A biopsy of damaged skin areas is performed for histological examination.
Microscopic examination of sputum.
Chest X-ray.
Lung perfusion scintigraphy is a study of pulmonary blood flow.
Spirometry.
Bronchoscopy.

The results of the study make it possible to identify the infection that caused hemosiderosis of the skin or other organs (if the disease has an infectious etiology).

Therapy for cutaneous hemosiderosis involves the prescription of drugs containing calcium, vascular strengthening agents, Rutin, Aescusan, ascorbic acid, and vitamins. If the disease develops against the background of an inflammatory process, therapy is supplemented with ointments with glucocorticoids.

Complications of skin hemosiderosis

In most cases, treatment of skin hemosiderosis has a favorable prognosis. Timely medical care allows you to avoid complications; at most, problems of an aesthetic nature arise without deterioration in well-being.
Other types of hemosiderosis can cause bleeding, dysfunction of cardio-vascular system, pulmonary hypertension and a number of other life-threatening conditions.

Prevention of skin hemosiderosis

The basis for preventing disruption of hemosiderin synthesis is timely treatment infectious, hematological and cardiovascular diseases. People prone to skin hemosiderosis should avoid blood transfusion complications(problems with blood transfusion), drug and chemical poisoning (intoxication).

Preventive measures are aimed at preventing diseases, causing disruption blood circulation of the lower extremities, since in some cases the provoking factors of hemosiderosis are injuries, hypothermia, and prolonged standing. Particular attention should be paid to health for those who, due to their professional activity I have to spend most of my time standing.

– a disease of the group of pigmentary dystrophies, characterized by excessive accumulation of the iron-containing pigment hemosiderin in the tissues of the body. There are local forms of the disease (cutaneous and pulmonary hemosiderosis) and general (with the deposition of hemosiderin in the cells of the liver, spleen, kidneys, bone marrow, salivary and sweat glands). Diagnosis of hemosiderosis is based on determining the iron content in the blood plasma, the total iron-binding capacity of the blood, as well as the pigment content in biopsy samples of tissues and organs. Depending on the clinical picture, treatment of hemosiderosis includes drug therapy (glucocorticoids, cytostatics, angioprotectors, vitamin C), in some cases plasmapheresis, and local PUVA therapy.

General information

Hemosiderosis is a disorder of the exchange of the hemoglobinogenic pigment hemosiderin with its local or systemic deposition in tissues. Hemosiderin is an iron-containing intracellular pigment formed during the enzymatic breakdown of hemoglobin. Hemosiderin synthesis occurs in sideroblast cells. Along with other hemoglobin derivatives (ferritin, bilirubin), hemosiderin is involved in the transport and deposition of iron and oxygen, and the metabolism of chemical compounds. Normal in small quantities hemosiderin is found in the cells of the liver, spleen, bone marrow, and lymphatic tissue. Under various pathological conditions, excessive formation of hemosiderin can occur with the development of hemosiderosis. Studying the epidemiology of hemosiderosis is difficult due to the wide variety of forms of this disorder.

Depending on the prevalence of the pathological process, local (local) and generalized (general) hemosiderosis are distinguished. Local hemosiderosis is the result of extravascular hemolysis, i.e., extravascular destruction of red blood cells. Local hemosiderosis can develop both in foci of limited hemorrhages (hematomas) and within an entire organ (lung, skin). Generalized hemosiderosis is a consequence of intravascular hemolysis - intravascular destruction of red blood cells under various common diseases. In this case, hemosiderin is deposited in the cells of the liver, spleen and bone marrow, giving the organs a brown (“rusty”) color.

Independent forms of hemosiderosis include:

hemosiderosis of the skin (Schamberg's disease, annular telangiectatic purpura of Majocchi, senile hemosiderosis, lichenoid pigmentosa and purpuric angiodermatitis, ocherous dermatitis, etc.)
idiopathic pulmonary hemosiderosis (brown induration of the lungs)

In the case where hemosiderin deposition is accompanied structural changes tissues and impaired organ function, they speak of hemochromatosis. Due to the variety of causes and forms of hemosiderosis, immunology, dermatology, pulmonology, hematology and other disciplines are studying it.

Causes of hemosiderosis

General hemosiderosis is a secondary condition, etiologically associated with diseases of the blood system, intoxications, infections, and autoimmune processes. Possible reasons excess hemosiderin deposition may serve hemolytic anemia, leukemia, liver cirrhosis, infectious diseases (sepsis, brucellosis, malaria, relapsing fever), Rh conflict, frequent blood transfusions, poisoning with hemolytic poisons (sulfonamides, lead, quinine, etc.).

The etiology of pulmonary hemosiderosis is not entirely clear. Assumptions have been made regarding hereditary predisposition, immunopathological nature of the disease, congenital disorders of the structure of the wall of the pulmonary capillaries, etc. It is known that patients with decompensated cardiac pathology (cardiosclerosis, heart defects, etc.) are more susceptible to the development of pulmonary hemosiderosis.

Cutaneous hemosiderosis can be primary (without previous skin lesions) or secondary (occurs against the background of skin diseases). The skin is predisposed to the development of primary hemosiderosis endocrine diseases(diabetes mellitus), vascular pathology (chronic venous insufficiency, hypertension). Dermatitis, eczema, neurodermatitis, skin trauma, and focal infection (pyoderma) can lead to secondary accumulation of hemosiderin in the layers of the dermis. Hypothermia, overwork, and taking medications (paracetamol, NSAIDs, ampicillin, diuretics, etc.) can provoke the onset of the disease.

Pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis is a disease characterized by repeated hemorrhages into the alveoli with subsequent deposition of hemosiderin in the pulmonary parenchyma. The disease primarily affects children and people young. The course of pulmonary hemosiderosis is characterized by pulmonary hemorrhages of varying intensity, respiratory failure and hypochromic anemia.

In the acute phase of the disease, patients experience moist cough with the release of bloody sputum. The consequence of prolonged or profuse hemoptysis is the development of severe iron deficiency anemia, pallor of the skin, icterus of the sclera, complaints of dizziness, and weakness. The progressive course of pulmonary hemosiderosis is accompanied by the development of diffuse pneumosclerosis, reflected by shortness of breath and cyanosis. Periods of exacerbation are accompanied by pain in the chest and abdomen, arthralgia, fever. Objective data are characterized by dullness of percussion sound, the presence of moist rales, tachycardia, arterial hypotension, spleno- and hepatomegaly.

During periods of remission, complaints are not expressed or absent, however, after each subsequent attack, the duration of clear intervals, as a rule, is reduced. In patients with chronic hemosiderosis, cor pulmonale; Severe infarction-pneumonia and recurrent pneumothorax often occur, which can cause death. Idiopathic pulmonary hemosiderosis can be combined with hemorrhagic vasculitis, rheumatoid arthritis, systemic lupus erythematosus, glomerulonephritis, and Goodpasture's syndrome.

Skin hemosiderosis

In the cutaneous version of hemosiderosis, deposition of iron-containing pigment occurs in the dermis. Clinically, the disease is characterized by the appearance on the skin of a hemorrhagic rash or pigment spots with a diameter of 0.1-3 cm. Fresh rashes have a brick-red color; old ones become brown, dark brown or yellowish color. Most often, pigmented lesions are localized on the skin of the ankles, legs, hands, and forearms; Sometimes the rash is accompanied by mild itching. In areas of skin lesions, petechiae, nodules, lichenoid papules, telangiectasia, and “rusty” plaques may appear.

Hemosiderosis of the skin has a chronic course (from several months to several years), and is more common in men aged 30-60 years. The general condition of patients with cutaneous hemosiderosis remains satisfactory; internal organs are not affected. The disease should be distinguished from atypical forms of lichen planus, pseudosarcoma and Kaposi's sarcoma.

Diagnosis of hemosiderosis

Depending on the form of hemosiderosis, its diagnosis can be carried out by a dermatologist, pulmonologist, infectious disease specialist, hematologist and other specialists. In addition to the physical examination, a general blood test, determination of serum iron, and total iron-binding capacity of the blood are performed. To identify hemosiderosis, the most important data are

Treatment of hemosiderosis

The first-line drugs used for the treatment of pulmonary hemosiderosis are glucocorticosteroids, but they are effective only in half of the cases. A method of combined treatment with immunosuppressants (azathioprine, cyclophosphamide) in combination with. In addition, symptomatic therapy is used: iron supplements, hemostatic agents, blood transfusions, bronchodilators, oxygen inhalations. In some cases, remission of the disease occurs after splenectomy.

Treatment of skin hemosiderosis includes local use of corticosteroid ointments, cryotherapy, ascorbic acid, rutin, calcium supplements, and angioprotectors. For severe skin manifestations of hemosiderosis, they resort to PUVA therapy and the prescription of deferoxamine.

Forecast and prevention of hemosiderosis

Idiopathic pulmonary hemosiderosis is a difficult to diagnose disease with a serious prognosis. The progressive course of the pathology leads to patient disability and the development of life-threatening complications - massive pulmonary hemorrhage, respiratory failure, pulmonary hypertension. The course of skin hemosiderosis is favorable. The disease is largely a cosmetic defect and tends to gradually resolve.

Prevention of hemosiderin metabolic disorders can be facilitated by timely treatment of skin and general infectious, vascular, hematological and other diseases, prevention of blood transfusion complications, and exclusion of drug and chemical intoxications.