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

Cutaneous manifestations of hemosiderosis.

Causes and risk factors Hemosiderin is formed by the breakdown of hemoglobin molecules by certain enzymes in sideroblast cells. Normally, a small amount of hemosiderin is found in the cells of lymphoid tissue, bone marrow, spleen and liver. With an increase in the synthesis of hemosiderin, 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, intoxication, 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 disorders of the immune system play an important role in the pathological mechanism of the development of hemosiderosis..

Forms of the disease According to the degree of spread of the pathological process, there are:.

general (generalized) hemosiderosis - it is caused by intravascular (endovascular) hemolysis of erythrocytes arising against the background of any systemic processes. Hemosiderin is deposited in the spleen, red bone marrow, 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 \u200b\u200bhematomas), and within an organ (for example, the lung).

Hemosiderin granules are distinguished according to the clinical course:.

hemosiderosis of the skin (ocher dermatitis, purpurous angiodermatitis, lichenoid pigment dermatitis, senile hemosiderosis, ring-shaped telangiectatic purpura of Mayocchi, Shamberg's disease);

pulmonary idiopathic hemosiderosis (brown induration of the lungs).

Pulmonary hemosiderosis progresses and leads to patient disability. It can be complicated by massive pulmonary bleeding, leading to rapid death..

In some cases, the deposition of hemosiderin 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, characterized by recurrent pulmonary hemorrhages of varying severity, increasing respiratory failure and persistent hypochromic anemia.

In the acute phase of the disease, there are:.

dyspnea;

chest pain;

cough with bloody sputum;

pallor of the skin;

icterus sclera;

cyanosis of the nasolabial triangle;

weakness;

dizziness;

increased body temperature.

Coughing up blood is one of the symptoms of hemosiderosis In 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. Chronic benign course. Brick-red pigment spots appear on the skin of the forearms, hands, legs and ankles. Over time, the color of the elements of the rash changes to yellowish, dark brown or brown. Damage to internal organs in the cutaneous form of hemosiderosis does not occur, the general condition does not suffer.

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