xanthoma
Introduction
Introduction to yellow tumor Xanthomatosis is a yellow or orange-yellow maculopapular rash or nodular xanthoma that occurs in the skin or tendon. Lipid-containing tissue cells and macrophages are localized in the dermis or tendon. Yellow, orange or brown-red papules, nodules, or plaques, yellow tumors. Often accompanied by abnormalities in blood lipids and other systems, a series of clinical symptoms appear. basic knowledge The proportion of illness: 0.007%--0.015% Susceptible people: no specific population Mode of infection: non-infectious Complications: Diabetes insipidus Coronary heart disease
Cause
Yellow tumor cause
Genetic factors (35%)
Mostly familial, due to congenital defects in lipid and lipoprotein metabolism, may be related to the degradation of lipoprotein lipase genetic activity or activity and affect lipoprotein breakdown.
Dietary factors (20%)
Dietary nutrition is caused by some mechanism. Secondary people are seen in atherosclerosis, hypothyroidism, diabetes, mucinous edema, nephrotic syndrome, pancreatitis, hepatobiliary disease, gout and other diseases, alcoholism, obesity and so on.
Drug factor (15%)
It can also be caused by estrogen therapy.
Pathogenesis
Causes yellow tumors with the following factors:
1. Increased plasma lipoprotein levels in most cases.
2. Abnormal blood vessel wall permeability.
3. Serum lipoproteins permeate the blood vessel wall and deposit connective tissue around the blood vessels.
4. The adventitial cells ingest and process lipoproteins, macrophages accumulate, and after lipoprotein exudation, these cells are transformed into vacuolated foam cells, serum lipoproteins are metabolized in foam cells, and foam cells are consistently contained. Corresponding lysosomal enzyme structure, newly occurring xanthoma has the same lipoprotein profile as blood, followed by cholesterol ester, cholesterol and phospholipid aggregation, some high lipoprotein xanthomas are reversible, according to the characteristics of blood lipids The World Health Organization classifies primary familial hyperlipoproteinemia into 5 types, the pathogenesis of which is type I: high chylomicronemia, lipoproteinase deficiency; type IIa: high -lipoproteinemia; IIb Type: high-beta lipoproteinemia and high pre-beta-lipoproteinemia V, sterol metabolism disorder; type III: wide beta-disease, high pre-beta-lipoproteinemia and high chylomicronemia, lipoprotein Metabolic disorder; type IV: high pre-beta-lipoproteinemia, disorder of glucose metabolism, alcoholism, gout; type V: combination of type I and IV, the incidence of different types of hyperlipoproteinemia is also very different, of which II Type is the most common, other according to the order of the number of cases For type IV, III, V, I, non-hyperlipoproteinemia xanthoma can be caused by abnormal serum proteins or abnormal proliferation of certain cells, such as macroglobulinemia, granulocyte and lymphocytic leukemia, Histiocytosis and the like.
Prevention
Yellow tumor prevention
Maintain good eating habits, pay attention to diet regulation, but intake high-fat, high-sugar, high-cholesterol foods. For people with a family history of genetics, blood lipids and serum lipoprotein levels should be detected early to prevent early detection.
Complication
Yellow tumor complications Complications diabetes insipidus coronary heart disease
Some patients with yellow tumors may also have mucosal damage, or even diabetes insipidus. The course of disease is long and can be relieved by themselves. It can also cause elevated blood lipids, accompanied by coronary heart disease.
Symptom
Yellow tumor symptoms common symptoms mucosal damage thyroid function hypothalamic pruritus itching urine collapse sputum granuloma
According to clinical and morphological characteristics, it is divided into the following types:
Primary high lipoprotein xanthomas
(1) flat yellow tumor: skin lesions are round or oval, flat and soft, light yellow or orange spots or slightly raised plaques, rice grains to 5cm size, occur in the eyelids, neck, torso, elbow, sputum The fossa, the medial side of the femur, the buttocks and the palms, the mucous membranes are not tired, and those who occur in the eyelids are called jaundice, and the symmetry occurs in the upper eyelids, single or multiple, slow development, which can affect the upper and lower eyelids on both sides, even Forming a yellow circle around the periorbital, more common in middle-aged women, about 25% of patients with hyperlipoproteinemia type II, showing elevated plasma cholesterol levels, occurring in the palm of the hand, can be uplifted stripe damage, known as palm Xanthomas, often a special symptom of type III hyperlipoproteinemia, generalized with less flat yellow tumors, flat orange or brownish yellow spots and plaques appearing symmetrically around the facial eyelids, on both sides of the neck, upper torso and Upper arm, generally seen in middle-aged and elderly people, has a good prognosis, and hyperlipoproteinemia may or may not be present.
(2) Nodular xanthoma: This type is often accompanied by type II and type III hyperlipoproteinemia. The early rash is a soft small papule or nodule, bright red or orange, gradually merging or increasing up to 2~ 3cm plaque, hard, surface hemispherical or lobulated, pedicled, slightly elastic, slightly painful, lack of symptoms or itching, good for stretching, such as elbow, knee, finger Joints and buttocks, etc., which occur in the tendon, are called jaundice, which are more common in the extension of the hands and feet, tendons and Achilles tendon. The lesions vary in size from soybeans and eggs, and the nodules are deep, smooth and firm. Different sizes, the surface of the skin can move as usual, and xanthomas almost always indicate a potential systemic disease.
(3) rash yellow tumor: often quickly become wholesale, distributed in the arms, limbs, face, chest, back and face, for needle or match head size papules, orange with red base, sometimes oral mucosa can also be involved After treatment, it can quickly disappear without leaving traces. Such rash is most common in type I hyperlipoproteinemia, III, IV, V-type hyperlipoproteinemia and secondary hyperlipidemia can be seen. .
2. Primary non-high lipoprotein xanthomatosis
(1) disseminated xanthoma: onset in adolescents, manifested as yellow, orange or brown yellow papules or nodules, scattered from the beginning to form a sheet, mainly distributed under the armpits, groin, neck, elbow, knee, etc. Large wrinkles in the body, basically symmetrical, about 1/3 of patients have mucosal damage, such as oral cavity, pharynx, larynx and even trachea, sometimes seriously affecting functions such as causing suffocation and life-threatening, about 1/3 of patients due to damage of hypothalamic-neuronal pituitary And cause temporary or mild diabetes insipidus, rash and diabetes insipidus can alleviate spontaneously, and the general prognosis is good.
(2) generalized flat xanthomatosis: more often after 40 to 50 years of age, most commonly in the periorbital period, can be concentrated in the medial malleolus, can also spread throughout the upper or even the entire eyelid, a wide range of symmetry can affect the torso And limbs, face, neck, upper torso and arm, the general prognosis is good, should pay attention to distinguish between xanthomatosis secondary to the reticuloendothelial system.
(3) juvenile yellow granuloma: also known as spastic yellow endothelial cell tumor, juvenile xanthomatosis, often within 6 months after birth, occasionally family history, lesions are single or multiple yellow or red needles to Pea size papules or nodules, round or oval, occur in the extremities of the extremities, face, scalp, trunk, can also affect the oral mucosa, eyes and internal organs, involving the visceral very few, serum lipids are not elevated, prognosis Good, most of them fade away after a few years or until the development of youth.
3. Secondary xanthoma
Refers to yellow tumors secondary to various causes, may be accompanied by increased blood lipids or normal blood lipids, often secondary to the following conditions: hypothyroidism, nephrotic syndrome, pancreatitis, diabetes, liver disease, hematopoietic diseases.
Examine
Yellow tumor examination
1. Determination of blood lipids, electrophoresis on paper
Can measure cholesterol, triglyceride, HDL, LDL, VLDL, etc., hyperlipoproteinemia type II, also known as familial hypercholesterolemia, is polygenic hypercholesterolemia, in xanthoma, xanthoma and knot Frequent xanthomas occur frequently, autosomal dominant inheritance, hyperlipoproteinemia type IV, also known as familial hypertriglyceridemia or familial combined hyperlipoproteinemia, in flat xanthomas and nodular yellow It often occurs in tumors.
2. Oral glucose tolerance test
Understand fasting and postprandial blood glucose levels to exclude hyperlipidemia and hyperlipoproteinemia caused by diabetes and impaired glucose tolerance (postprandial hyperglycemia).
3. Liver function, kidney function, thyroid function test
Understand albumin, total protein and immunoglobulin levels to exclude secondary xanthomatosis caused by diseases such as biliary cirrhosis, nephrotic syndrome and mucinous edema.
4. Histopathology: Xanthoma is a reaction tumor. The microstructure of different types of xanthoma is similar. There is no need for differential diagnosis. The main manifestations are lymphocytes and tissue-like cells around the peripheral blood vessels of the xanthoma. These cells contain lipids and are sliced. During the fixation process, the lipids are dissolved and the cytoplasm is foamy. The foam cells are filled with macrophages that phagocytose lipids, which are the main components of xanthoma. In addition, there are characteristic giant cells with polygonal arrangement in a ring shape. That is, Touton cells, the ultrastructure of phagocytic lipids include vacuoles containing lipoproteins, birefringent cholesterol crystals and phospholipids, and phospholipids exhibit intracellular myelin images; additional fatty acids and neutral fats, other characteristics Including rash-type xanthoma with consistent inflammation and longer-lasting xanthoma with fibrotic increased fibroblastic response, it is necessary to note that when foam cells or lipids are seen in the tissue, leprosy, deep fungus is excluded. Disease, histiocytoma and progressive lipid necrosis.
Diagnosis
Yellow tumor diagnosis
According to the relatively solid yellow, orange pimples and nodular lesions can be initially diagnosed as yellow tumors, with emphasis on distinguishing between primary and secondary, primary hyperlipoproteinemia or non-hyperlipoproteinemia Xanthomatosis, pay attention to the presence or absence of systemic diseases, especially cardiovascular, pancreatic, liver, endocrine, hematopoietic system lesions, determination of blood lipids is essential, histopathology has an auxiliary diagnostic value.
It should be differentiated from gout. The latter lesions mainly occur in the joints and cartilage tissues. They are hard knots of normal skin color, often accompanied by arthritis, and the pain in the affected area is obvious. It is differentiated from calcinosis. The latter has a hard papule knot. It is characterized by a knot or a lump, and can be broken to discharge the oily substance of the cheese, and the wound is healed by the scar.
