multicentric reticulohistiocytosis
Introduction
Introduction to polycentric reticular cell hyperplasia Multicentric reticulo-histiocytosis (also known as reticular cell granuloma, reticular cell hyperplasia, etc.). The basic feature is that the skin or mucous membrane has single or multiple papules or nodules, accompanied by joint lesions, sometimes combined with systemic or local symptoms and signs, typical tissue like tissue cells and multinucleated giant cell infiltration. basic knowledge Sickness ratio: 0.05% Susceptible people: no special people Mode of infection: non-infectious complication:
Cause
The cause of polycentric reticular cell hyperplasia
(1) Causes of the disease
The etiology of this disease is still unclear. About one-fifth of the patients have tuberculosis, so it is considered to be related to tuberculosis infection, but most patients have no tuberculosis, tuberculosis culture is negative, and some people suspect that it is related to viral infection, but biopsy tissue culture The virus was not isolated.
(two) pathogenesis
The pathogenesis of this disease is still not very clear, this disease is a granulomatous lesion, mainly involving the skin and joints. In the tissue cells and giant cells of this disease, lipid deposition can be seen. In a few cases, yellow tumor rash and blood cholesterol can be seen. It may be mildly or temporarily elevated, so some people think that this disease is a special abnormality of lipid metabolism. Because these changes are not universal, and serum and intracellular lipids are often inconsistent, some people negate this view. It is believed that phagocytic lipids are only a follow-up phenomenon. Under electron microscopy, a large number of dense lysosome-like bodies are found in the cytoplasm of most tissues. These bodies are strongly positive for acid phosphatase, lysozyme and naphthalene oxidase. It may be a substance that can produce a variety of enzymes. Experiments have shown that artificially activated tissue cells also contain a large number of lysosomes, and the released proteases and hydrolases can cause tissue destruction and inflammation similar to this disease. Electron microscopy also shows that The cells contain intact or degraded collagen fibers, but they are non-specific changes, so some people think that the disease may be induced by certain unknown factors. Nucleated cells or giant cells, synovium synovial cells (A, B type) Evolution of cell hyperplasia, formation of granulomatous changes.
Pathology: The disease has the following histopathological features:
1 There are a large number of multinucleated giant cells in the lesion;
2 fibrous tissue hyperplasia;
3 lipid deposition;
4 A large number of eosinophilic ground glass-like tissue cells, the typical changes are most obvious in muscle and lymphoid.
Prevention
Multicentric reticular cell hyperplasia prevention
1. Reduce or avoid pathogenic factors and prevent viral infections.
2. Pay attention to exercise and increase the body's ability to resist disease.
Complication
Multicentric reticular cell hyperplasia complications Complication
One-fourth of the cases are complicated with malignant tumors. The tumor can occur in the breast, uterus, ovary, stomach, colon, larynx, lung, etc. It can also be lymphoma and leukemia. Of these cases, 73% are diagnosed with this disease. After the tumor occurs, it can also combine multiple organ damage.
Symptom
Multicentric reticular cell hyperplasia symptoms common symptoms rheumatoid arthritis tissue cell hyperplasia bone destruction joint deformity papule joint swelling
The disease is seen in adults, mostly in women, the earliest manifestations of skin and joint symptoms, can occur sequentially or simultaneously.
1. Skin: All patients have skin damage, mainly papules and nodules, hard texture, brownish red or light yellow, common in the upper limbs, frontal, neck, upper torso, generally small and large number of papules, The nodules are larger and the number is smaller, the papules and nodules can be mixed, and the rashes in different parts are slightly different. In almost all cases, there are hand and finger rashes, and the sides of the interphalangeal joints are more common. The knee and forearm extension are large, infiltrating deep solitary nodules and tumor-like changes. The forearm is mainly small papules, scattered or fused to mossy, and the face and trunk are mainly miliary papules. Like red rash on hair.
2. Mucosa: Half of the patients can see mucosal papules, the most common in the lips and tongue, followed by the buccal mucosa and gums, and the pharynx and cornea can also be rash, but less common, the lips are papules and nodules of varying sizes. The tongue is mainly papules, the buccal mucosa is a red papule, and sometimes it can be a blister-like nodule.
About 1/4 of the cases can be seen as a yellow tumor-like rash, the number is generally more, after a certain period of time can self-resolved, but often relapse.
3. Joint lesions: mostly symmetrical polyarthritis, can cause joint deformity and dysfunction, the earliest manifestation of bilateral symmetry hand small arthritis, similar to rheumatoid arthritis, proximal interphalangeal joint, metacarpophalangeal joint The wrist and the wrist are swollen and sweaty, and the distal interphalangeal joint can also be affected. In addition, the cervical vertebrae, the temporomandibular joint and the knee joint are also common sites of lesion invasion. When the onset is insidious, the lesion can develop as a joint without symptoms. Disability, especially in the hands and feet, recurrent carpal tunnel syndrome, palmar fascia fibroproliferative hand contracture, is also a common bone and joint manifestations of this disease, early visible joint red, swollen, hot, pain, sometimes With regard to uterine effusion, abnormalities often occur after the acute phase.
Examine
Examination of polycentric reticular cell hyperplasia
Half of the patients had anemia and a slight increase in erythrocyte sedimentation rate. One third of patients had a mild increase in transient cholesterol, and the ratio of serum albumin and globulin was reduced or inverted.
X-ray examination, in the early stage of the lesion, the joint space is widened, and there is a small amount of effusion. Further development of the joint surface bone destruction, joint space narrowing and joint deformity, a few patients with chest radiographs may have nodular or diffuse Invasive lesions.
Diagnosis
Diagnosis and diagnosis of multicentric reticular cell hyperplasia
Mainly according to single or multiple papules and nodules of the skin, accompanied by symmetrical polyarthritis, early acute changes accompanied by redness, swelling, heat, pain, bone destruction and malformation in the future, combined with pathology, a large number of forms Singular tissue cells and giant cells, but without mitosis and deep nuclear staining, can diagnose the disease.
The disease should be distinguished from rheumatoid arthritis, abnormal fat metabolism, histiocytosis and malignant lymphoma. Although the joint symptoms of this disease and rheumatoid arthritis are very similar in clinical practice, the latter is rarely extensive. Skin lesions, the histological changes of the two are also very different, because the disease can be seen in the pathologically many foam cells (glassy-like cells), clinically visible yellow tumor rash, it needs to be distinguished from lipid metabolic diseases, joint deformity and Pathologically, there are a large number of various forms of tissue cells and multinucleated giant cells, which are rare in metabolic diseases. The rash of this disease sometimes resembles histiocytosis X and malignant lymphoma, but there are obvious differences in pathology. In particular, the cell morphology is singular, but mitosis and nuclear deep staining are not obvious.
