Acute Posterior Multifocal Squamous Pigment Epitheliopathy
Introduction
Brief introduction of acute posterior multifocal squamous pigment epithelial lesions Acute posterior multifocal squamous epithelial lesion (acute multifocal posterior placoid pigmentepitheliopathy, AMPPPE) is an inflammatory disease that occurs mainly at the level of retinal pigment epithelium and choroidal capillaries. basic knowledge The proportion of illness: 0.0002% - 0.00055% Susceptible people: no special people Mode of infection: non-infectious Complications: retinal vasculitis, cystoid macular edema, optic discitis, retinal vein occlusion, subretinal neovascular membrane
Cause
Causes of acute posterior multifocal squamous pigment epithelial lesions
(1) Causes of the disease
The cause is unknown, but often combined with systemic diseases, some patients with tuberculin test is significantly positive, or adenovirus, Lyme disease infection history, there are also increased HLA antigen (HLA-B7, HLA-DR2); some patients are suffering from AMPPPE has systemic fever, headache, dizziness, muscle and joint pain, or symptoms of upper respiratory tract infection, lymphadenopathy, abdominal discomfort, papules, etc., all of which suggest the possibility of infection. I have suspected measles virus, hepatitis B virus, Bacterial infections play a role in their occurrence; others believe that allergic reactions to different pathogens or antimicrobial agents are involved in the disease.
(two) pathogenesis
The pathogenesis is still not fully understood. It is generally believed that delayed type hypersensitivity plays an important role in the occurrence of the disease. Antigen-specific CD4 Th1 cells secrete various cytokines such as -interferon, IL-2, lymphotoxin, etc. It can attract and activate macrophages, neutrophils, natural killer cells, and stimulate the differentiation of CD8 T cells. These activated cells and their secreted cytokines can exert various biological effects, and finally cause choroidal vasculitis and retinal pigmentation. Inflammation of the epithelium.
The primary affected part of the disease is still controversial. Some people think that the primary site of inflammation is in the retinal pigment epithelium, which may also include photoreceptors. Some scholars believe that the primary site of the disease is in the choroid, and allergic reactions cause choroidal capillaries. The choroidal arterioles before lobular occlusion are occluded, causing secondary retinal pigment epithelial changes.
Prevention
Prevention of acute posterior multifocal squamous pigment epithelial lesions
Prevention of a cold may be effective in some patients.
Complication
Complications of acute posterior multifocal squamous pigment epithelial lesions Complications retinal vasculitis macular cystic edema optic disc retinal vein obstruction subretinal neovascular membrane
Can be complicated by retinal vasculitis, cystoid macular edema, optic discitis, retinal vein occlusion and subretinal neovascularization.
Symptom
Acute posterior multifocal squamous pigment epithelial lesions common symptoms pigmentation erythema nodules
The acute phase is mostly in the posterior pole, but also as far as the mid-week. There are many gray-white or cheese-colored flat lesions, which are located under the retina. The shape is white, such as scaly, larger; the Chinese are mostly irregular or non-regular. It is round and the lesions can be fused together. The overall impression is smaller than that of white people. After 1 to 2 weeks, the lesions gradually disappear, entering the scar stage, with pigmentation and pigment accumulation. There are new lesions, one after another, which can last for several months. There may be retinal detachment on the surface of the lesion, but it rarely occurs. In addition, it may be accompanied by venous dilation, para-venous exudation; optic disc edema, optic discitis and optic neuritis, 50% The patient has inflammatory cells in the vitreous, the degree of variation is very large, most of them are mild; individual patients even have central retinal vein occlusion, and the rare late complication is choroidal neovascular membrane, which is an important cause of permanent damage to central vision. Department can also see iridocyclitis, upper scleritis and so on.
APMPPE patients may be associated with a variety of systemic diseases such as nodular erythema, localized enteritis, hepatomegaly, adenovirus V-type infection, thyroiditis, lymphoma, cerebrovascular disease, cerebrospinal lymphocytosis and protein elevation, nodules Disease, mycobacterial infection, microvascular nephropathy, platelet aggregation abnormality, hearing loss, PPD (pure protein derivate) skin test negative.
Examine
Examination of acute posterior multifocal squamous pigment epithelial lesions
In some patients, the tuberculin test was significantly positive, and there was also an increase in HLA antigen (HLA-B7, HLA-DR2). The white blood cell count was routinely examined to rule out the possibility of infection.
1. Visual field examination Some patients have a central dark spot or a side dark spot.
2. Fluorescein fundus angiography fluorescein fundus angiography is important for the diagnosis of this disease. In the acute phase, active lesions show early weak fluorescence, medium-term shows persistent weak fluorescence in inflammatory lesions, and late can appear strong Fluorescence and staining, this strong fluorescence lasts for about 30 minutes, caused by the diffusion of fluorescein from the choroid to the retinal pigment epithelium or the diffusion of fluorescein between the damaged retinal pigment epithelium. The pigment epithelium is atrophied, and the depigmented area exhibits typical transflective fluorescence accompanied by salt-and-salt mottled fluorescence without fluorescein leakage.
3. Indocyanine green angiography examination of active lesions showed early and late weak fluorescence, large choroidal vessels were visible in the early weak fluorescence zone, and the boundary of weak fluorescence damage was clear in the later stage, usually irregular, after the lesion healed, The early and late stages also showed choroidal weak fluorescence, but the range was smaller than that of active lesions, and the degree of weak fluorescence was also lower than that of the acute phase.
4. Electrophysiological examination of the retinal current map, electro-oculogram examination showed that most patients had no abnormal changes, but in some patients there may be sub-normal changes in the electroretinogram and electro-oculogram, these changes can completely disappear after the disease is recovered.
Diagnosis
Diagnosis and differentiation of acute posterior multifocal squamous pigment epithelial lesions
According to the patient's medical history and clinical manifestations, combined with clinical trials and auxiliary examination results, the diagnosis can be basically determined.
All diseases that can cause multifocal lesions are identified.
1. Multifocal choroiditis is more common in women, 25% of single eyes, grayish yellow lesions in the fundus, varying in size, distributed in the posterior pole and peripheral parts, 50% combined with anterior uveitis, vitreous may have inflammation, acute lesions after healing Left a significant pigmented imprinted scar, retinal detachment above the active lesions, often suggesting choroiditis below, fluorescein angiography may show strong fluorescence of optic disc edema and cystoid macular edema.
2. The average age of the shotgun-like chorioretinopathy is 50 years old, many eyes are sick, many women, the fundus is oval, milky yellow, flat, and the spotted lesions of the border are scattered from the optic disc along the retinal vessels. Such as bird bullets, less weeks, can be combined with optic disc edema, retinal vasculitis.
3. Multiple sexual white spot syndrome is more common in young women, single eye disease, history of colds before the disease, multiple scattered white spots on the fundus, small can be less than 50m, can be as large as 500m, generally smaller than the shotgun The spots were smaller than the APMPPE squamous lesions. The lesions were distributed from the posterior pole to the mid-peripheral part, concentrated in the posterior pole and the optic disc, which caused the expansion of the physiological blind spot, and the granular pigment epithelial changes were observed in the center of the macula.
4. Claudication choroidal lesions (or choroiditis) often involve both eyes, which occur successively. The lesions start from the optic disc and show chronic progressive progression. If the macula is involved, the visual acuity is seriously impaired, and deep scars are formed after healing, than AMPPPE. The lesion is deep, and the recurrence is found in the immediate vicinity of the lesion; the recurrence of AMPPPE disease is multifocal, and it can cause pigmentation and scar after healing. In this case, it needs to be differentiated from the retinal degeneration of the blanket, but the AMPPPE disease is generally no disc shrinkage, blood vessels. The diameter of the tube is normal and not fine, and the ERG is normal or close to normal. The ERG and EOG examinations of this disease can be normal or below normal even in the acute phase.
