Sunset-like or sunset-like eyes

Introduction In the late stage of the field of exudative choroiditis, the choroidal pigment cells and retinal pigment epithelial cells are severely damaged and lost. The fundus is red when the sun sets, and it is called the evening glow or the eve of the fundus.

Cause

Reasons for the evening glow or the evening sun:

(1) Causes of the disease

The etiology of Vogt-Koyanagi-Harada syndrome is not fully understood, mainly related to autoimmune response and infection factors.

(two) pathogenesis

The pathogenesis of this disease is still not very clear. It may be that cell immunity and humoral immunity work together to cause disease.

1. Cellular immunity causes tissue damage: this damage is mediated by lymphocytes. The experiment confirmed that the lymphocytes of patients with this disease were sensitized by melanocyte surface antigen, and the sensitized lymphocytes attacked melanin as a target cell. That is to say, melanocytes are both antigens of immune response and target cells that are damaged by attack by sensitized lymphocytes. Antibodies against various components of the pigmented membrane have been detected from patients, the most important of which are antibodies against melanocyte surface antigens. The antibody destroys melanocytes by an anti-dependent cell-mediated cytotoxic mechanism, indicating that it is autoimmune caused by humoral immunity.

According to Sugiura, the disease is a melanocyte-specific autoimmune disease. The antigen that induces this autoimmunity is located on the surface of melanocytes. In normal people, since the antibody immunosurveillance system functions, the immune-active cells do not undergo an immune attack on their own melanocytes, and this state is called immune tolerance. In the case of this disease, the immune tolerance to autologous melanocytes may be terminated by the following two factors: 1 primary surveillance of immune function; 2 changes in melanocytes, antigens on the cell surface Sex is modified.

2. The role of immunogenetics in pathogenesis: Many autoimmune diseases are known to be closely related to human leukocyte antigen (HLA). Sugiura Qingzhi detected HLA-A, B, and D locus antigens in a group of patients. The frequency of HLA-BW54 antigen was 45.2%, the control group was 13.2%, the LD-Wa antigen was 66.7%, and the control group was 16%. The relative risk of HLA-BW54 was 4.9, and that of LD-Wa was 10.5, that is, the incidence of carrying these two antigens was 4.9 times and 10.5 times that of non-carriers, respectively. HLA-BW54 and LD-Wa are HLA-B and D-site antigens, respectively. These two antigens are not found in whites, and thus are considered to be unique antigens in the Far East. The disease is more common in Japan and the East, but less common in European and American whites, which also shows that the disease is closely related to immune genetics. Ohno also confirmed that the relative risk of DR4 and MT3 in patients with this disease increased by 15.2 and 74.5 times, respectively, compared with the control. This disease, like other autoimmune diseases, is also closely related to HLA-D (DR) site antigen (MT3). All cases with D (DR) site antigen were positive for MT3, indicating that the disease is highly correlated with immunogenetic factors. DR4 and MT3 are also antigens unique to Japanese and Far East people. See the related effects of Vogt-Koyanagi-Harada syndrome and HLA.

3. Pathology: The typical pathological change of this disease is choroidal histology: the lesion is a nodular granuloma lesion formed by lymphocytes, plasma cells surrounded by epithelioid cells and multinucleated giant cells, and there is no necrotic lesion in the center. Epithelioid cells are large cells with clear cytoplasm and contain many organelles, lysosomes and phagosomes. Melanin particles are visible in the phagosome. There is a Delen-Fuchs nodule that protrudes inward into the choroid. This nodule is composed of degenerated retinal pigment epithelial cells and epithelioid cells. The pathological changes of the iris ciliary body are essentially the same as the choroidal changes. They are lesions composed of epithelioid cells, lymphocytes and plasma cells. Sometimes there are signs of mitosis of lymphocytes, but in the iris, epithelioid cells are inferior to the choroid. Visible inside.

Corneal epithelial melanocytes and melanin particles are reduced, while Langhan cells are increased. Normal Langhan cells are only found in the superficial layer, and the disease can also be seen in the basal layer.

The pathological changes of the skin are the same as those of the corneal epithelium, that is, the melanocytes and melanin particles are reduced, and the Langhans cells are increased. This cell is also found in the basal layer. A small amount of lymphocytes and mild inflammatory cell infiltration were observed in the epidermis. There is no melanocytes in the dermis, but melanocytes derived from the mother's plaque can be seen in the hips of the Mongolian plaque, and there is a fusion of melanocytes with lymphocytes, which is completely seen in the pigmented membrane. the same. Infiltration of cells in the dermis is very light, no epithelial-like cells are formed, and occasionally lymphocytic infiltration is accompanied by epithelioid cells. In addition to Langhan cells, there are still identical cells in the dermis with rod-shaped granulosa cells, which have active migratory and phagocytic functions.

According to the observed characteristics of melanocytes, they can be divided into superficial and deep types. The melanocytes in the pigmented membrane, meninges, inner ear and dermis belong to the deep type, while the melanocytes in the corneal epithelium and epidermis belong to the superficial type. The characteristics of the two types of melanocytes are significantly different, that is, the deep melanocytes lose the function of synthesizing melanin. Under electron microscope, the cell wall of this type of cells is thin and the basement membrane is incomplete. On the contrary, superficial melanocytes have active melanin synthesis. The cell membrane has no deep basement membrane features.

an examination

Related inspection

Fundus examination, fundus fluorescein angiography, fundus examination, and CT examination of the temporal region

Examination and diagnosis of the evening glow or the evening fundus:

1. Lumbar puncture: Lumbar puncture and cerebrospinal fluid examination is a useful auxiliary laboratory test, but it is not widely used in clinical applications. This is because in most patients, the diagnosis can be confirmed based on medical history, clinical examination, and fluorescein fundus angiography. The patient's cerebrospinal fluid changes mainly manifested as lymphocytosis. In one week after the onset of inflammation, cerebrospinal fluid lymphocytosis occurred in about 80% of patients, and 97% of patients had this change in 1 to 3 weeks. Cerebrospinal fluid lymphocytosis usually disappears within 8 weeks. When the inflammation recurs, there is no longer a cerebrospinal fluid lymphocytosis, so there is no diagnostic value for this examination of patients with chronic prolonged uvitis and recurrent uveitis.

2. Immunological examination: Vogt-Koyanagi-Harada disease can cause a variety of immunological abnormalities, such as antibodies against uveal, anti-photosensitive extracellular, anti-retinal S antigen, anti-Müller cells, etc. in serum. Serum IgD levels and -interferon levels were also elevated in patients. But these changes are not specific, so they are of little value in determining the diagnosis. HLA antigen typing of patients found that HLA-DR4, HLA-DRw53 antigen positive, helpful for diagnosis.

3. Fluorescein fundus angiography: Fluorescein fundus angiography is of great value in the diagnosis of Vogt-Koyanagi-Harada disease. The changes in angiography can be very different at different stages of the disease.

Differential diagnosis

Differential diagnosis of the evening glow or the eve of the fundus:

A differential diagnosis should be made with other fundus abnormalities:

1. Fundus punctate or flaming hemorrhage: Fundus hemorrhage is not an independent eye disease, but a feature common to many eye diseases and certain systemic diseases. Common in retinopathy caused by hypertensive retinopathy, diabetes and kidney disease. Retinal vein inflammation, retinal vein occlusion, optic disc vasculitis, and blood diseases cause retinopathy, ocular traumatic fundus hemorrhage. The same pathological damage, such as retinal hemorrhage, exudation, microangioma, neovascularization, etc., due to various causes.

Due to the complicated etiology, the disease has a long course of disease and is prone to repeated attacks, which seriously affects vision and causes many serious complications. Such as macular degeneration (macular cystic edema, macular degeneration), neovascular glaucoma, vitreous hemorrhage, optic atrophy, proliferative retinopathy, traction retinal detachment, if not timely and effective treatment, often can lead to blindness.

2. The macula is found to have gray discoloration in the fundus, and Austin-type children with cerebral sulphurosis, also known as Austin-type metachromatic leukodystrophy, is a joint disease of cerebral sulphur disease and mucopolysaccharidosis. It is characterized by mild Hurler syndrome, multiple bone dysplasia, severe neurological symptoms and markedly low intelligence. Under the fundus examination, it can be found that the macula is grayish and discolored, and even blind.

3. The red spot on the macula of the fundus, Niemann-Pick's disease (NPD) is an inherited metabolic disease caused by sphingomyelin and cholesterol deposited in various organs of the body. It has the main features of liver, splenomegaly, red spot on the macula of the fundus and large foam-like cells in the bone marrow smear. The disease was first reported by Niemann in the first case in 1914. In 1922, Pick described the pathological findings in detail, hence the name. For the first time, China reported 2 cases in 1963, and there have been reports in the following.

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