diffuse fasciitis
Introduction
Introduction to diffuse fasciitis Diffuse fasciitis (EF) is a rare disease characterized by diffuse swelling and sclerosis of the fascia. Known as "diffuse fasciitis with high gamma-globulinemia and eosinophilia", patients do not have elevated eosinophils in the peripheral blood or tissue infiltration and high gamma globulin Hemorrhage, and therefore some scholars believe that it should be called "sclerosing fasciitis" to better reflect the pathological features of the disease. basic knowledge The proportion of sickness: 0.8% Susceptible people: no special people Mode of infection: non-infectious Complications: pleural effusion, pericardial effusion, synovitis, proteinuria, aplastic anemia, thrombocytopenic purpura, leukemia
Cause
Causes of diffuse fasciitis
(1) Causes of the disease
The etiology is still unclear, but according to the patient's clinical manifestations, especially hypergammaglobulinemia, increased circulating immune complexes, low complement, some patients also have low titers of anti-nuclear antibodies and rheumatoid factor, peripheral blood And eosinophils in the fascia tissue increased and infiltrated, sensitive to corticosteroid treatment and may be associated with some autoimmune diseases, so it is believed that the disease is related to immune abnormalities.
(two) pathogenesis
1. Schulman (1974) proposed an immune-mediated theory for this disease. The evidence supporting this theory is: 1 high gamma-globulin (usually IgG) is present; 2 in some patients' fascia IgG and complement deposition; 3 occasional increase of bone marrow plasma cells, he also suggested that exercise and its secondary tissue damage, can lead to the release of autoantigen, the result can cause an allergic reaction to fasciitis, acidophilia Granulocyte chemokines can increase the number of cells, or eosinophils and neutrophil infiltration represent a chronic inflammatory response, in addition, eosinophilic fasciitis can sometimes be combined with autoimmune hemolytic anemia The fact that aplastic anemia also indirectly supports the immunological pathogenesis of this disease.
2. The pathological changes of pathology are fascial inflammation, hypertrophy, edema and fibrosis attached to the muscle. The diseased fascia is loosely attached to the medial side of the subcutaneous fat.
In order to fully understand the pathological changes of the disease, the biopsy specimens must include the epidermis, dermis, fascia and muscles. The epidermis is generally normal, but in a few cases, the epidermis is slightly atrophied and the epidermal cell processes disappear. These are usually seen later in the course of the disease. Changes, accompanied by varying degrees of dermal reticular layer hardening, in terms of dermal reticular layer hardening, various aspects of the report are quite inconsistent, Barnes reported that 2 / 3 cases have mild to extensive dermal reticular sclerosis, and other scholars reported The dermis is normal. In the subcutaneous fat, fibrous trabeculae are arranged along the interlobular septa. In the subcutaneous tissue near the fascia, lymphocytes, plasma cells, histiocytes and eosinophils are generally found to be diffuse and perivascular. Inflammatory infiltration, the same infiltration is seen in the hypertrophic and fibrotic fascia, and eosinophil infiltration in the tissue is a variability finding, although this finding is not directly proportional to blood eosinophils. However, sometimes eosinophil infiltration in the tissue may be accompanied by a significant increase in eosinophils in the peripheral blood. In a few cases, polymorphism is seen in the subfascial muscles. Sexual cell infiltration, which is distributed around the blood vessels and between the muscle bundles. Sometimes it can be accompanied by muscle fiber degeneration and regeneration as seen in dermatomyositis or polymyositis. Direct immunofluorescence can be found in the fascia. IgG, IgM, C3 are calm.
Prevention
Diffuse fasciitis prevention
1. Remove the inducement of the disease, pay attention to hygiene, strengthen physical exercise to improve immunity and prevent infection.
2. Early diagnosis and early treatment, do not give up treatment easily when the disease is relieved.
Complication
Diffuse fasciitis complications Complications pleural effusion pericardial effusion synovitis proteinuria aplastic anemia thrombocytopenic purpura leukemia
This disease can occur in the lesions of joint contracture and dysfunction, pleural effusion, pericardial effusion, polyarticular synovitis, proteinuria, etc., may also be associated with aplastic anemia, thrombocytopenic purpura, cycle Atrophic neutropenia, leukemia, etc.
Symptom
Diffuse fasciitis symptoms Common symptoms Muscle sore shoulder and back muscle pain Skin sclerosis Low fever fascia pain Lack of eosinophilia Protein urinary extremities flexed orange... Carpal tunnel syndrome
The disease occurs in autumn and winter, 30% to 50% of patients have overwork, trauma, cold and upper respiratory tract infections, etc., the onset is sudden, the first symptoms are swelling of the limb skin, hardening and tightness or both skin erythema And physical activity is blocked, followed by joint or muscle soreness, fatigue or fever, etc., about 25% of patients have irregular fever during the course of the disease, with low fever.
1. Skin lesions The first part of the lesion is the lower limbs, especially the lower end of the lower leg, followed by the forearm, thigh, a few from the back of the foot, the waist is onset, the lesions are affected by the limbs, followed by the trunk, the hands and feet can sometimes occur, the face , refers to, the toe is rarely involved, the damage is characterized by subcutaneous deep tissue sclerosis swelling, the edge is unclear, the surface of the affected area is uneven, the orange-like appearance when lifting the limb, and the groove along the direction of the superficial vein Depression, due to the deeper position of the disease, the skin above it can be unaffected or affected, so the skin color, skin texture and texture can be normal, can be moved or pinched, sometimes pigmentation, the disease can start It is diffuse or lumps, and later developed into diffuse or strip-like, nodules are rare, generally no Raynaud's phenomenon, fingertip ulcers and perivascular telangiectasia, only individual patients may be scleroderma-like lesions.
2. Exogenous skin manifestations This disease may involve the tendon sheath, collagen hyperplasia and fibrosis, so that the tendon sometimes has a strong hard cord shape, and the forearm flexor tendon is involved in the wrist flexion and extension difficulty and finger movement disorder and the like carpal tunnel syndrome. Performance, there may be joint pain, muscle soreness and tenderness, especially the gastrocnemius muscle is more common, the skin lesions can also undergo joint contracture and dysfunction through the joint.
3. System damage obvious visceral damage is rare in EF, if it is very mild, individual organs that can be involved include lung, esophagus, thyroid, bone marrow, liver, spleen, heart, kidney, etc., pleurisy, pericarditis may occur , polyarticular periostitis, proteinuria, etc., there are reports of EF with aplastic anemia, thrombocytopenic purpura, Grave disease, Sjogren's syndrome, Hashimoto's thyroiditis, rheumatoid arthritis, Hodgkin's disease and even Turned into lupus and so on.
Examine
Examination of diffuse fasciitis
1. Blood routine red blood cell and platelet counts can be slightly reduced, and eosinophils increase in about 47.6% of cases.
2. About half of the patients with erythrocyte sedimentation rate increase, if there is a hematologic disorder, the corresponding blood cell abnormalities and bone marrow abnormalities, occasionally proteinuria.
3. Blood biochemical and immunological examination ANA positive rate was 30.8%, anti-dsDNA antibody was 33.3% positive, RF36.4% positive, -globulin increased by 73.3%, IgG, IgA, IgM were 60%, 26.7%, 20%, respectively Increased, CIC 85.7% positive.
4. Histopathology: At present, the diagnosis of EF mainly depends on histopathological examination. The biopsy of the disease should reach the depth of muscle and fascia. The EF lesion is mainly in the fascia, which is characterized by collagen fibrosis, thickening and fibrosis, and collagen is transparent. , glassy or homogenized, focal lymphocytes around the blood vessels, tissue cells and plasma cells infiltration, varying amounts of eosinophil infiltration, vasodilation and hyperplasia, proliferating collagen tissue in the fascia can be extended In the subcutaneous fat lobules interval, some of the fat lobules are wrapped in the sclerotic lesions, and can also affect the underlying muscles, inflammation of the superficial muscles occurs, lymphocytes around the muscle bundles, plasma cells and eosinophils infiltrate, a few Cases of dermis may also have the above mild lesions; the epidermis is normal, and a small number may have mild atrophy and basal pigment cells.
Direct immunofluorescence of the skin has IgG, C3 deposition in the fascia and muscle compartment, IgG, C3 deposition around the blood vessels in the deep and subcutaneous fat, and IgM deposition in the dermal epithelial junction, but these changes are non-specific, It does not help the diagnosis of EF.
Diagnosis
Diagnosis and diagnosis of diffuse fasciitis
Diagnostic criteria
The diagnosis is based on: excessive fatigue, trauma, cold history, acute onset, skin sclerosis in the limbs and trunk, and epidermis, dermis only slightly involved or normal, and the surface of the lesion is uneven when the limb is lifted. It can be seen that there is a sulcus-like depression along the superficial vein, no Raynaud phenomenon, the visceral is not tired or involving light, the peripheral blood eosinophils increase, histopathology shows fascia thickening with varying amounts of eosinophil infiltration.
Differential diagnosis
1. Scleroderma needs to be differentiated from localized scleroderma and systemic scleroderma (extremity). Localized scleroderma is characterized by three stages of skin undergoing localized swelling, followed by hardening and finally atrophy. Skin sclerosis disappears, skin is absent, dry and sweat-free, touch hard and tough, systemic scleroderma (extremity) is mostly from the extremities, facial skin begins to edema swelling and hardening, and limb sclerosis To the elbow, the lower third of the knee joint stops, almost all have Raynaud's phenomenon, common perivascular telangiectasia and fingertip ulcer, and often accompanied by visceral involvement, the most commonly involved organs are the esophagus and lung, leading to the esophagus Peristalsis is weakened, pulmonary interstitial fibrosis, etc., regardless of localization or systemic scleroderma, its histopathological changes are mainly in the dermis and epidermis.
2. Adult scleredema often occurs in the neck, and later spread to the face, trunk, and finally the limbs or the limbs are not involved, the damage is wider, the skin is hard and non-depressed, can not be lifted, often before the disease Infectious diseases and other infection history, histopathology showed thickening of the dermis, swelling of the collagen fibers, homogenization, widening of the gap, filled with an acidic mucopolysaccharide matrix.
3. Dermatomyositis has a wide range of muscles and severe symptoms. It is mainly composed of the scapular band and the proximal limb muscles. The upper eyelid has edematous purple-red spots and the back of the hand, the Gottron sign of the phalanx, the serum muscle enzymes such as CPK, LDH and AST and 24h creatinine excretion significantly increased.
