Primary cryoglobulinemia is more common in young people and middle-aged people, and is slightly more common in women than in men. When the patient’s body surface temperature drops in cold, cold globulin in the limb blood vessels becomes precipitated or jelly-like, blocking the capillaries and causing ischemic necrosis and vasospasm of the blood vessel wall, skin purpura and cold urticaria are most common, and some patients may have Raynaud’s phenomenon. How to check the cold globulin precipitation or jelly-like?
1, cold globulin determination: the patient’s serum will be placed at 4 ℃ for at least 12h, individual cases even need 1 week to precipitate the cold globulin for detection. It is <10mg/L in normal people, while it is often above 250mg/L in patients with this disease. 2.Immunoglobulin determination: IgM is often increased, and some IgG and IgA are increased. 3.Complementation measurement: Type I is normal, and complement is often decreased in mixed type. 4.Hematological examination: blood sedimentation is often accelerated, blood coagulation is abnormal, and platelets are reduced. 5. Histopathology: no significant changes in epidermis. Eosinophilic amorphous homogeneous proteinaceous deposits in small vessels of dermis and subcutaneous tissue, characterized by fibrin-like necrosis and infiltration of neutrophil granulocytes with perivascular consolidation. The most common skin lesion is hemorrhagic purpura, which mostly starts in the lower extremities and gradually extends to the femur, perineum, and buttocks, and rarely extends to the upper extremities and oral mucosa. The rash is characterized by papules, petechiae, petechiae, small nodules, and in severe cases, blisters, macules, ulcers, and gangrene. Itching or burning may be conscious, and in severe cases, pain may be present. Next, cold urticaria, Raynaud’s phenomenon, reticular cyanosis, vasospasm of the extremities, and cyanosis are seen. Hyperpigmentation is seen in chronic cases. Arthralgia is a prominent symptom in patients with mixed cold globulinemia and is commonly seen in the hand and knee joints. Renal damage may manifest as acute or chronic nephritis, and may even lead to renal insufficiency or renal failure and death. The main manifestations of the nervous system are peripheral neuropathy manifested as abnormal sensation, numbness, motor impairment, and loss of tendon reflexes. Central nervous system involvement is rare. Other symptoms include hepatosplenomegaly, abdominal pain, pericarditis, and generalized lymph node enlargement. The diagnosis of the disease is based on clinical manifestations and a significant increase in serum cryoglobulin. It should be noted that cryoglobulins are not easily detected after treatment with corticosteroids or after the improvement of the existing concomitant diseases. A temporary increase in cryoglobulins should also be excluded.