Disseminated eosinophilic collagen disease

Introduction to disseminated eosinophilic collagen disease The disease is an allergic disease characterized by fever, respiratory symptoms, hepatosplenomegaly, and increased peripheral blood eosinophils, first reported by Engfeldt and Zettertröm in 1956. basic knowledge Sickness ratio: 0.0001% Susceptible people: no specific people Mode of infection: non-infectious Complications: erythema, yellow tumor, splenomegaly

Causes of disseminated eosinophilic collagen disease

(1) Causes of the disease

The exact cause is still unclear. Because of eosinophilia, asthma, and elevated serum IgE, it may be a type I hypersensitivity-mediated disease.

(two) pathogenesis

The pathogenesis is still not clear, it may be a type I hypersensitivity mediated disease, from the joint muscle pain, nodular polyarteritis-like vasculitis, the disease is similar to rheumatism, so type III hypersensitivity Important pathogenic effects, as to how type I and type III hypersensitivity reactions synergistically cause disease, remains to be studied.

Pathology: microscopic epidermal hyperkeratosis, partial parakeratosis, irregular extension of the epidermis, etc., not all cases have epidermal changes, but there are dermal changes, manifested as loose edema of dermal collagen fibers, shallow levels of limitations Fibrin changes, from the superficial to the middle layer of the dermis, there are moderate eosinophils and tissue cells around the capillaries, small blood vessels and sweat glands, a small amount of lymphocytes and plasma cells infiltrate, and the organs of the whole body are mainly vasculitis and Interstitial inflammation, some organs showed granuloma with necrosis and eosinophil infiltration. The histopathological changes of this disease have the following characteristics:

1 fibrous connective tissue-like fibrin degeneration and fibrin-like necrosis;

2 vasculitis, mainly small vessel disease;

3 more mature eosinophils diffuse infiltration and granuloma formation.

Disseminated eosinophilic collagen disease prevention

1. Eliminate and reduce or avoid the disease factors, improve the living environment, develop good living habits, prevent infection, pay attention to food hygiene, and rational diet.

2. Pay attention to exercise, increase the body's ability to resist disease, do not over-fatigue, excessive consumption, quit smoking and alcohol, maintain balance, and overcome anxiety and tension.

3. Early detection and early diagnosis of the primary disease, establish confidence in the fight against disease, adhere to treatment.

Disseminated eosinophilic collagen disease complications Complications, erythema, yellow tumor, spleen

The disease can be complicated by joint cavity effusion; joint deformation; muscle weakness; erythema, erythema multiforme; papules; dermatitis, plaque, yellow tumor-like damage; necrosis, scar; erythroderma, transient pneumonia, bronchopneumonia, heart Expand the liver and spleen.

Disseminated eosinophilic collagen disease symptoms Common symptoms Nausea constipation Abdominal pain Relaxation High intracranial high pressure wheal dyspnea Diarrhea Joint swelling

Mainly manifested as fatigue, body aches and discomfort, poor appetite, irritability and insomnia, etc. This disease, like other collagen diseases, also has the characteristics of multiple system damage.

1. Fever has almost all cases of fever, and more parallel with the activity of the lesion, can be relaxation heat, but also for heat retention, body temperature is more than 38 °C.

2. The joint symptoms are polyarthritis or joint pain, sometimes there is joint fluid accumulation, joint swelling, joint symptoms are light and heavy, heavy activity is limited, morning stiffness, but most do not cause joint deformation, body joints are Can be affected, but more common in the limbs, especially the hand joints are most susceptible, and some cases are accompanied by muscle pain and muscle weakness.

3. Skin lesions have the following types of skin changes:

1 erythema, erythema multiforme;

2 pimples;

3 dermatitis, plaque, yellow tumor-like damage;

4 necrosis, scars;

5 erythroderma.

A rash may exist alone or in combination at the same time. The miliary papules on the frontal and trunk are the most common skin changes in the early stage. As the disease progresses, it gradually spreads to the limbs and turns into sputum. Measles-like erythema, wheal or atopic dermatitis-like rash, accompanied by intense itching, the beginning of miliary papules can also be quickly converted into body redness, thickening and desquamation, often accompanied by itching, so-called A dermatological rash, sometimes accompanied by a mossy appearance of the limbs, leaving a fine reticular pigmentation after the lesion has subsided, and the lesion may also initially appear as a clear erythema scattered around the body, the size of which is Eggs or palms are not equal, the edges are bright red, the center is lighter in color, and the skin is slightly higher. The erythema is mixed with light brown pigmentation, often merging with each other to form plaque or yellow tumor-like changes. Necrosis, scarring after dissipation, different types of skin lesions may occur in different parts of the body, such as erythema in the trunk, erythematous exudative erythema-like damage, and limbs in the miliary Rash or atopic dermatitis-like rash, in short, the disease has a diversity of skin changes, but the constant characteristic is disseminated, often accompanied with itching.

4. Cardiopulmonary symptoms During the onset of the disease, most patients have respiratory symptoms, which are manifested by varying degrees of cough, sometimes as asthma-like dyspnea, coughing foamy sputum or mucus sputum, and eosinophils in sputum smear due to Lung tissue has eosinophil infiltration, can cause transient pneumonia symptoms, due to long-term use of glucocorticoid treatment can reduce the body's resistance, it is easy to secondary bacterial infection, and finally lead to bronchial pneumonia, making the disease worse and become the disease The cause of death.

More than half of the patients have enlarged heart, but early asymptomatic, congestive heart failure can occur later, almost all cases have sinus tachycardia, heart rate and body temperature separation.

5. Digestive symptoms can show abdominal pain, nausea, vomiting, occasional diarrhea or constipation, or alternating between the two, which may be caused by gastrointestinal eosinophil infiltration, about half of the patients have liver, splenomegaly .

6. Some cases of neurological symptoms may have neurological symptoms, such as gait disturbance, disturbance of consciousness, visual impairment, soft palate of the extremities, drooping of the eyelids, intracranial hypertension and pathological reflex.

7. About 1/3 of other patients have swollen lymph nodes, which can be swollen systemic lymph nodes, or local lymphadenopathy, especially in the neck and axillary lymph nodes.

Detection of disseminated eosinophilic collagen disease

1. The blood routine and the total number of leukocytes can be increased, and the classification counts only have eosinophils elevated, mostly mature, and there is more ESR in the active period.

2. Urine routine can have mild proteinuria, microscopic hematuria or tubular urine, and urine changes are generally mild.

3. Biochemical examination may have mild abnormalities in liver function, and protein electrophoresis may show an increase in gamma globulin.

4. Immunological examination All cases have IgE, IgG increased, 2 / 3 of patients with rheumatoid factor positive, about 1 / 5 of patients anti-nuclear antibody positive, E-rosette formation number and PHA and other markers of cellular immunity test How low is, and the complement is reduced.

5. X-ray examination of chest X-ray has different degrees of inflammatory infiltration, sometimes visible heart enlargement, individual cases may have hand deformities, joint cavity narrowing, bone destruction, occasionally visible joint dislocation or subluxation.

Diagnosis and differentiation of disseminated eosinophilic collagen disease

The diagnosis of this disease mainly depends on skin lesions, clinical manifestations, pathological features and peripheral blood eosinophilia, combined with multi-system involvement characteristics, can be diagnosed.

Differential diagnosis

1. Nodular polyarteritis is mainly caused by visceral damage, especially heart, kidney and nervous system symptoms. The lesions are mainly purple spots or nodules distributed along the arteries, and there are no diffuse pruritic miliary papules or Plaque, pathological changes in fibrous necrosis, arteritis, mostly no epidermis, dermal collagen fiber changes, rheumatoid factor and anti-nuclear antibody negative, these pathological and laboratory abnormalities are different from disseminated eosinophils Cellular collagen disease.

2. Allergic granuloma is a multi-system organ involving granulomatous vasculitis, mainly pulmonary infiltration, manifested as paroxysmal asthma, peripheral blood eosinophilia, skin lesions mainly skin or subcutaneous knot Section, purple spot, pathological changes to fibrin-like necrotic vasculitis, mainly involving the muscular layer arteries, accompanied by granulomatous changes, no changes in skin collagen fibers, rheumatoid factor, anti-nuclear antibodies are negative.

3. Wegener's granuloma is a kind of necrotizing granulomatous vasculitis, mainly involving the respiratory tract, kidney and eyes, often with upper respiratory tract bone destruction, no pruritic miliary papules, plaques, no skin collagen fiber changes. The anti-nuclear antibody was negative and the IgE level was normal.

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