Alpha1-antitrypsin deficiency panniculitis

Introduction to 1-antitrypsin deficiency lipitis 1-1-antitrypsindeficiency associated with paniculitis, reported in 1972 as a subtype of nodular panniculitis, 1-antitrypsin is a polypeptide glycoprotein synthesized by hepatocytes It is an inhibitor of serum protease, which can affect trypsin, pancreatic elastase, urokinase, skin collagenase, polynuclear cell neutral protease, coagulation co-factor, etc. The function of these enzymes can regulate metabolism and inhibit inflammation. basic knowledge The proportion of illness: 0.0006% Susceptible people: no specific people Mode of infection: non-infectious Complications: pleural effusion phlebitis

The cause of 1-antitrypsin deficiency panniculitis

(1) Causes of the disease

A large amount of fatty acid that may cause inflammation in fat cells in the absence of 1-antitrypsin.

(two) pathogenesis

1-antitrypsin is a polypeptide glycoprotein synthesized by hepatocytes and is an inhibitor of serum protease. Its action can affect trypsin, pancreatic elastase, urokinase, skin collagenase, polynuclear cell neutral protease, coagulation Cofactors, etc., the function of these enzymes can regulate metabolism and inhibit inflammation.

1-1-antitrypsin deficiency panniculitis prevention

Every night, use warm water to soak your feet to the whole body. At the same time, massage your ears, comb your hair, replenish your kidneys, and pass through the meridians.

1-1-antitrypsin deficiency lipitis complications Complications pleural effusion phlebitis

If a common pleural effusion or thrombophlebitis occurs due to trauma, about 1 in 5 patients have pulmonary infarction.

1-1-antitrypsin deficiency lipid inflammatory symptoms common symptoms pigmented lesion abscess subcutaneous nodule nodules

Similar to nodular panniculitis, the skin may have micro-lifted liquefied nodules or abscess formation, can be ulcerated, such as trauma due to common pleural effusion or thrombophlebitis, about 1/5 of patients have lung Infarction.

Examination of 1-antitrypsin deficiency panniculitis

Histopathology: invasive inflammatory nodules of subcutaneous adipose tissue, mainly by mononuclear cells and neutrophil infiltration, accompanied by macrophages, which resemble early changes of nodular panniculitis, and later fibrosis and fat necrosis or ulceration form.

Diagnosis and differentiation of 1-antitrypsin deficiency panniculitis

According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.

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