eosinophilic fasciitis
Introduction
Introduction to eosinophilic fasciitis Eosinophilic fasciitis (eosinophilic fasciitis) is a connective tissue disease involving the deep fascia of the limbs and a scleroderma-like manifestation, also known as diffuse fascia with hypergammaglobulinemia and eosinophilia. Inflammation, eosinophilic diffuse fasciitis, and Shulman syndrome. The disease may be a subcutaneous type of localized scleroderma. It is rare in clinical practice. Mainly manifested as a sudden onset of skin lesions, scleroderma-like skin damage, and peripheral blood eosinophils significantly increased. basic knowledge The proportion of illness: 0.01% (the age of disease is 30-60 years old. The incidence of the disease is about 0.01%) Susceptible people: no special people Mode of infection: non-infectious Complications: pericardial effusion
Cause
Causes of eosinophilic fasciitis
The etiology of this disease has not yet been clarified. At present, it is believed that its pathogenesis is related to immune abnormalities. For example, some patients may have hypergammaglobulinemia, increased circulating immune complexes, low complement, rheumatoid factor positive, and immunoglobulin between fascia. Deposition or concomitant with some autoimmune diseases, which support this disease is an immune disease. For the attribution of this disease, it is considered that this disease is a type of scleroderma spectrum, that is, between localized hard Between skin disease and systemic scleroderma, some patients can be converted to systemic scleroderma during the course of the disease, but most scholars believe that it is not a scleroderma, an independent disease.
Prevention
Eosinophilic fasciitis prevention
The key to preventing this disease is to avoid overworked and traumatic cold. For patients with restricted activities, they should strengthen their physical therapy and supplement with physical therapy.
Complication
Eosinophilic fasciitis complications Complications
Common complications of this disease:
1, joint or muscle soreness, fatigue, low fever and so on.
2, joint contracture and dysfunction.
3. Fibrous peripheral neuropathy.
4, there are reports of lung, esophagus, liver, spleen, thyroid and bone marrow can also be affected, such as pericardial effusion, obstruction-based mixed ventilatory disorders.
Symptom
Eosinophilic fasciitis symptoms Common symptoms Fascial pain Subcutaneous deep fascia... Muscle soreness, shallow fasciitis, joint contracture, low heat fatigue
More common in men, the ratio of male to female is 2..1, the age of onset is more than 30-60 years old, often have a history of overwork before the onset, strenuous exercise, trauma, cold and upper respiratory tract infection may also induce the disease.
Often the limbs are swollen, taut, stiff, or have both erythema and joint activity limitation. The lower extremities of the lesions are more common in the lower leg, followed by the forearm, a few from the thigh, waist Abdominal or insteps and other diseases, 95% of the patients involved in the limbs, 48% of the hands and feet, 43% of the trunk, the face is usually not affected, the damage is characterized by subcutaneous deep tissue swelling, marginal confinement or pervasation Unclear, when the affected limb is lifted, the surface of the lesion is uneven, and a pit-like depression can be seen along the direction of the superficial vein. The skin of the affected area can be pinched, the texture is normal, and the pigmentation can be accompanied by different degrees.
Generally no obvious systemic symptoms, a small number of patients may be associated with joint or muscle soreness, fatigue, hypothermia, etc., lesions involving joints can cause joint contracture and dysfunction, may also be associated with fibrous peripheral neuropathy, reported lung, esophagus, liver The spleen, thyroid and bone marrow can also be affected. Echocardiography detects cases of pericardial effusion, and pulmonary function tests have mixed ventilatory dysfunction showing obstruction.
Examine
Examination of eosinophilic fasciitis
The blood red blood cell count is slightly reduced, the white blood cell count is normal, the eosinophils are significantly increased, the erythrocyte sedimentation rate is increased, the gamma globulin is increased, the IgG and/or IgM is increased, the circulating immune complex is increased, the rheumatoid factor, and the antinuclear antibody are positive. Serum sarcoplasmic enzymes CPK and AST increased slightly. The 24-hour urinary creatine output was slightly elevated. The micro-circle microcirculation examination showed that the tube sputum became shorter, the deformity branch increased, the site dome was congested, and the blood flow was granular. Slower, blood viscosity increases.
Diagnosis
Diagnosis and identification of eosinophilic fasciitis
diagnosis
According to the specific subcutaneous deep tissue of the affected area, the skin has a linear depression consistent with the direction of the superficial vein, accompanied by local soreness. In combination with pre-injury, it is often caused by overwork, trauma, cold, etc. It is not difficult to diagnose. Perform a disease biopsy to assist in diagnosis.
Differential diagnosis
The disease sometimes needs to be differentiated from adult scleredema, the latter often starts in the neck, and then spreads to the face, trunk, and finally involves the upper and lower limbs; the lesion is diffuse non-depressed swelling, hard; often before the onset History of upper respiratory tract infection; histopathology showed that collagen fibers were swollen and homogenized, and the gap was filled with acid mucopolysaccharide matrix. In addition, the disease needs to be differentiated from dermatomyositis. The latter muscle lesions are often referred to as the scapula and the proximal extremities. Main; upper eyelids have edematous purplish red spots and hand back, Gottron sign of phalanx back; serum sarcoplasmic enzymes such as CPK, LDH and AST and 24-hour urinary creatine excretion increased significantly.
