Hemosiderosis is a disease of the pigmented dystrophy group 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 form) 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 the determination of the iron content in blood plasma, the total iron binding capacity of blood, as well as the pigment content in tissue and organ biopsies. Depending on the clinical picture, the treatment includes drug therapy (glucocorticoids, cytostatics, angioprotectors, vitamin C), in some cases plasmapheresis, local PUVA therapy.
Meaning
Hemosiderosis is a violation of the exchange of hemoglobinogenic pigment hemosiderin with its local or systemic deposition in tissues. Hemosiderin is an iron-containing intracellular pigment formed during the enzymatic cleavage of hemoglobin. Hemosiderin synthesis occurs in sideroblast cells. Along with other hemoglobin derivatives (ferritin, bilirubin), hemosiderin participates in the transport and deposition of iron and oxygen, the metabolism of chemical compounds. Normally, hemosiderin is found in small amounts in the cells of the liver, spleen, bone marrow, and lymphatic tissue. In various pathological conditions, excessive hemosiderin formation may occur with the development of hemosiderosis. The study of 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 form can develop both in foci of limited hemorrhages (hematomas) and within the whole organ (lung, skin). Generalized hemosiderosis is a consequence of intravascular hemolysis – intravascular destruction of red blood cells in 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”) coloring.
Independent forms include:
- hemosiderosis of the skin (Shamberg’s disease, annular telangiectatic purpura Majocchi, senile hemosiderosis, lichenoid pigmented and purpurous angiodermitis, ocher dermatitis, etc.)
- idiopathic pulmonary hemosiderosis (brown lung induration)
In the case when the deposition of hemosiderin is accompanied by structural changes in 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 engaged in its study.
Causes
General hemosiderosis is a secondary condition etiologically associated with diseases of the blood system, intoxication, infections, autoimmune processes. Possible causes of excessive hemosiderin deposition may be hemolytic anemia, leukemia, cirrhosis of the liver, infectious diseases (sepsis, brucellosis, malaria, recurrent typhus), rhesus conflict, frequent hemotransfusion, poisoning with hemolytic poisons (sulfonamides, lead, quinine, etc.).
The etiology of lung hemosiderosis is not completely clear. Assumptions are made regarding hereditary predisposition, the 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 hemosiderosis of the lungs.
Cutaneous hemosiderosis can be primary (without previous skin lesions) or secondary (occurs against the background of skin diseases). Endocrine diseases (diabetes mellitus), vascular pathology (chronic venous insufficiency, hypertension) predispose to the development of primary hemosiderosis of the skin. Dermatitis, eczema, neurodermatitis, skin injuries, focal infection (pyoderma) can lead to secondary accumulation of hemosiderin in the layers of the dermis. Hypothermia, fatigue, taking medications (paracetamol, NSAIDs, ampicillin, diuretics, etc.) can provoke the onset of the disease.
Hemosiderosis of the lungs
Idiopathic pulmonary hemosiderosis is a disease characterized by repeated hemorrhages in the alveoli with subsequent deposition of hemosiderin in the pulmonary parenchyma. The disease mainly affects children and young people. The course of hemosiderosis of the lungs is characterized by pulmonary bleeding of varying intensity, respiratory failure and hypochromic anemia.
In the acute phase of the disease, patients have a wet cough with the release of bloody sputum. The consequence of prolonged or copious hemoptysis is the development of severe iron deficiency anemia, pallor of the skin, icteric sclera, complaints of dizziness, weakness. The progressive course of hemosiderosis of the lungs is accompanied by the development of diffuse pneumosclerosis, which is reflected by dyspnea and cyanosis. Periods of exacerbation are accompanied by chest and abdominal pain, arthralgia, fever. Objective data are characterized by dulling of percussion sound, the presence of wet wheezing, tachycardia, arterial hypotension, splenic and hepatomegaly.
During periods of remission, complaints are not expressed or absent, but after each subsequent attack, the duration of light intervals, as a rule, is reduced. In patients with chronic hemosiderosis, a pulmonary heart is often formed; severe infarction-pneumonia, recurrent pneumothorax, which can cause death, often occur. Idiopathic hemosiderosis of the lungs can be combined with allergic purpura, rheumatoid arthritis, systemic lupus erythematosus, glomerulonephritis, Goodpasture syndrome.
Hemosiderosis of the skin
In the cutaneous variant of hemosiderosis, the deposition of iron-containing pigment occurs in the dermis. Clinically, the disease is characterized by the appearance of hemorrhagic rash or pigment spots on the skin with a diameter of 0.1-3 cm. Fresh eruptions have a brick-red color; old ones acquire a brown, dark brown or yellowish color. Most often, pigmented foci are localized on the skin of the ankles, shins, hands, forearms; sometimes rashes are accompanied by a slight itching. Petechiae, nodules, lichenoid papules, telangiectasia, plaques of a “rusty” color may occur in places of skin lesion.
Hemosiderosis of the skin is chronic (from several months to several years), 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.
Diagnostics
Depending on the form of hemosiderosis, its diagnosis can be carried out by a dermatologist, a pulmonologist, an infectious disease specialist, a hematologist and other specialists. In addition to the physical examination, a general blood test is performed, the determination of serum iron, the total iron-binding capacity of the blood. To detect hemosiderosis, the most important data are biopsies of tissues (skin, liver, lung, bone marrow), histological examination of which reveals the deposition of hemosiderin. As a diagnostic test, a desferal test is used – determination of the amount of iron in the urine after intramuscular administration of 500 mg of deferoxamine. With a content of more than 1 mg of iron in the urine, the sample is considered positive.
In case of hemosiderosis of the lungs, chest X-ray, CT, perfusion scintigraphy of the lungs, microscopic examination of sputum, spirometry are additionally performed. Conducting bronchoscopy with the taking of washing waters allows you to detect siderophages and erythrocytes in lavage fluid.
Treatment
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, cyclophosphane) in combination with plasmapheresis is proposed. In addition, symptomatic therapy is used: iron preparations, hemostatic agents, hemotransfusion, bronchodilators, oxygen inhalation. In some cases, remission of the disease occurs after splenectomy.
Treatment of hemosiderosis of the skin includes the local use of corticosteroid ointments, cryotherapy, ascorbic acid, rutin, calcium preparations, angioprotectors. In severe cutaneous manifestations of hemosiderosis, PUVA therapy and the appointment of deferoxamine are resorted to.
Prognosis and prevention
Idiopathic pulmonary hemosiderosis is a difficult to diagnose disease with a serious prognosis. The progressive course of pathology leads to disability of the patient, the development of life–threatening complications – massive pulmonary hemorrhage, respiratory failure, pulmonary hypertension. The course of hemosiderosis of the skin is favorable. The disease is more of a cosmetic defect and is prone to gradual resolution.
Timely therapy of skin and general infectious, vascular, hematological and other diseases, prevention of hemotransfusion complications, exclusion of medicinal and chemical intoxications can contribute to the prevention of hemosiderin metabolism disorders.