Kimura disease
Introduction
Introduction to Kimura's disease Kimura's disease (Kimura's disease), also known as angiolymphoid hyperplasia with eosinophilia (angiolymphoid hyperplasia with eosinophilia), is an inflammatory lesion with abundant blood vessels, endothelial cell proliferation and a large number of lymphocytes and eosinophils. Asians are prone to this disease, which occurs in the skin of the head and neck, and can also affect the eyelids. basic knowledge Sickness ratio: 0.0001% Susceptible people: no specific population Mode of infection: non-infectious Complications: bronchial asthma
Cause
Cause of Kimura disease
(1) Causes of the disease In patients with skin lesions, some patients were found to have bronchial asthma and elevated serum IgE. In patients with glomerulopathy, IgE was found to be deposited in the lesion, and some patients had eosinophils in the surrounding blood. Increased cells indicate abnormal immune function and may be an allergic disease.
(2) The pathogenesis of pathogenesis is unknown.
Prevention
Kimura disease prevention
There is no effective preventive measure for this disease. Early detection and early treatment are the key to prevention.
Complication
Kimura disease complications Complications, bronchial asthma
About 12% of patients with KD have nephrotic syndrome and a few have bronchial asthma.
Symptom
Symptoms of Kimura's disease Common symptoms Nodules, ocular protrusion, ptosis, and lacrimal gland enlargement
The onset age ranged from 38 to 72 years old, with an average age of 51 years. The skin lesions in the head and neck were purple-blue. The borders were unclear and nodules or masses on the surface of the skin. The eyelid skin and conjunctiva were involved, showing single or multiple purple-blue knots. Section, indicating that there are more blood vessels and inflammatory infiltration in the lesions, the lesion mainly involves the lacrimal gland, the lacrimal gland is swollen when the lacrimal gland is invaded, the upper eyelid is red and swollen near the lacrimal gland, and the clinical symptoms are similar to the lacrimal gland inflammation; but most of the lesions are located above the eyelids, forming a lump. For the eyeball to protrude, the mass can invade the upper iliac muscle to make the ptosis; the upper rectus muscle invades the eyeball, and sometimes the lumps adhere to the periosteum. The orbital lesions generally do not involve systemic lesions. Choroidal lesions may be associated with Head and neck skin nodules, CT examination revealed lacrimal gland, extraocular muscle enlargement.
Examine
Kimura disease check
Immunological examination
Mainly for the examination of immune indicators, providing a reference for disease diagnosis (Figure 4).
2. Pathological examination
The naked eye sees the lesion as red or brown, the boundary of the mass is unclear, there are lymphocytes in the connective tissue around the lesion, and the lymphoid follicles are associated with the germinal center. There are more small blood vessels in the lesion, mainly capillaries and venules. Small arteries, swelling of endothelial cells, vacuoles in the nucleus, pleats of the nuclear membrane, eosinophilic cytoplasm, formation of large vacuoles, mimic pseudocavity; hypertrophic endothelial cells can protrude into the lumen, but also through damage And inflamed blood vessel wall protruding to the surrounding tissue, there are more eosinophils between the vascular space, there are plasma cells, mast cells and lymphocytes infiltration, the lesion may have fresh or old bleeding, generally no extensive fiber There was no necrosis in the diseased tissue, and no mitosis was observed. The continuous pathological section showed immature blood vessels originating from the arteries. The wall of the artery proliferated and extended to the surrounding connective tissue. The larger blood vessels often had inflammation of the wall and the elastic layer was interrupted. And destruction, there is mucopolysaccharide deposition in the endometrial region, the lymph node is invaded, the structure of the lymph nodes exists, there are most lymphoid follicles, and there is a large amount of eosinophilic fine between the follicles. Cells, blood vessels increased; invading the lacrimal gland, there may be many lymphoid follicles of different sizes, there are a large number of eosinophils and blood vessels between the follicles, vascular endothelial cells are swollen, electron microscopic examination of endothelial cells is rich in filaments, similar Actin, more mitochondria, a little Weibel-Palade body, the plasma membrane is invaded into the vesicle, the surface of the cell has microvilli protruding into the cavity, and the outer layer of endothelial cells and pericytes are surrounded by multiple layers of basement membrane material.
CT can be found in the lacrimal gland, and the extraocular muscles are enlarged.
Diagnosis
Diagnosis of Kimura disease
diagnosis
Eyelid skin, conjunctiva with purple-blue nodules or eyeball protrusions, may consider the possibility of this disease, can be used for CT examination showed lacrimal gland, extraocular muscle swelling, sputum can be seen in the border, density is not homogeneous mass Shadow, but it is indistinguishable from other masses in the sputum. Pathological examination of the skin, subconjunctival or intraorbital mass can confirm the diagnosis. In patients with systemic disease, the peripheral blood eosinophil count is increased.
Differential diagnosis
CT and MRI are not easy to distinguish this disease from malignant tumors, lymphomas and hemangiomas.
