Rare diseases, accompanied by bleeding

Bleeding caused by the pathology of blood vessels

Bleeding can be the result of platelet abnormality, coagulation factors and blood vessels. Diseases of the bleeding of the vessels are caused by the pathology of the vascular wall and are usually manifested by petechiae, purpura, but rarely cause serious bleeding. Bleeding may be the result of a deficiency in vascular or perivascular collagen in Ehlers-Danlos syndrome, other rare hereditary connective tissue diseases (for example, pseudoxanthoma elasticum osteogenesis imperfecta Marfan syndrome.) Bleeding may be the main manifestation in scurvy or purple Shanlein-Genoh, allergic vasculitis, In patients with bleeding diseases of blood vessels, hemostasis tests remain, as a rule, within the limits of normal .The diagnosis is based on clinical manifestations.

[1], [2], [3]

Autosensitivity to erythrocytes (Gardner-Diamond Syndrome)

Autosensitivity to erythrocytes is a rare disease that occurs in women. Characterized by localized pain and painful ecchymosis, manifested mainly on the limbs.

In women with autosensitivity to erythrocytes, intradermal injection of 0.1 ml of autologous erythrocytes or erythrocytic stroma can cause pain, swelling and sealing of the injection site. This result suggests that the red blood cells penetrating the tissues are involved in the pathogenesis of the lesion. However, in most patients there are also manifestations of severe psychoneurotic syndrome. Moreover, psychogenic factors, such as self-induced purpura, may have a connection with the pathogenesis of the syndrome in some patients. The diagnosis is based on the study of the place of intradermal injection of auto-erythrocytes and the place of control injection (without erythrocytes) 24 and 48 hours after injection. Damage can complicate the interpretation of the test, so for injections it is necessary to choose places that are hard to reach for the patient.

[4], [5], [6]

Dysproteinemia that cause vascular hemorrhagic rash (purpura)

Amyloidosis causes the deposition of amyloid in the vessels of the skin and subcutaneous tissues, which can cause increased fragility of the vessels, leading to purpura. In some patients, amyloid adsorbs the blood clotting factor X, causing its deficiency, but this usually does not lead to the development of bleeding. Periorbital hemorrhages or hemorrhagic rash developing in a patient after a light stroke in the absence of thrombocytopenia suggest the presence of amyloidosis.

Cryoglobulinemia is caused by immunoglobulins, which precipitate when the plasma is cooled during its passage through the skin and subcutaneous tissues of the extremities. Monoclonal immunoglobulins formed in Waldenstrom's macroglobulinemia or multiple myeloma sometimes behave like cryoglobulins, mixed immune complexes IgM-IgG, formed in some chronic infectious diseases, more often with hepatitis C. Cryoglobulinemia can cause inflammation of small vessels, which is manifested by purpura. The presence of cryoglobulins can be detected using laboratory tests.

Hypergammaglobulinemic purpura is vascular purpura and is found primarily in women. Recurrent, small, palpable, hemorrhagic skin lesions are localized on the lower limbs. These formations leave small residual brown spots. Many patients have manifestations of other immunological diseases (for example, Sjogren's syndrome, SLE). Diagnostic finding is a polyclonal increase in IgG (diffuse hypergammaglobulinemia in the electrophoresis of whey proteins).

The blood viscosity increase syndrome is the result of a significant increase in the plasma IgM concentration and can cause purpura and other forms of pathological hemorrhage (eg, profuse nasal bleeding) in patients with Waldenstrom macroglobulinemia.

[7], [8], [9]