scleredema
Introduction
Introduction to scleredema Scleredema is an unexplained, usually benign, rare disease, also a systemic rheumatism characterized by sudden onset of systemic symmetry, severe edema, induration, lesions and systemic scleroderma edema Similar changes occur, the disease usually relieves itself within 6 to 12 months, but it can last for decades. Curzio (1775) first reported the disease, and Pifford (1876) discussed its clinicopathological features in more detail. Bushkle (1902) identified it as a special independent disease. basic knowledge The proportion of illness: 0.005% Susceptible people: no special people Mode of infection: non-infectious Complications: acute renal failure cardiogenic shock
Cause
Cause of scleredema
Disease factors (35%):
The content of acid mucopolysaccharide was significantly increased in the patient's skin, and it was thought to be due to the increased concentration of hyaluronic acid in the skin of the lesion. A considerable number of patients were secondary to the original diabetes infection, indicating the immunity after infection. The reaction is related to the pathogenesis of this disease, and it also suggests that glucose metabolism disorder plays a certain role in the pathogenesis of this disease.
Autologous hormone factor (25%):
It has been suggested that pituitary function and peripheral nerve abnormalities play an important role in the pathogenesis. Local injection of estrogen can induce non-depressed edema, which is very similar to scleredema, suggesting that the body is stimulated by sudden stress and the adrenal cortex is stimulated. An estrogen-like steroid hormone that causes skin changes.
Body mechanism factors (35%):
Some people believe that the disease is related to the allergic reaction after streptococcal infection, but it needs to be further demonstrated because some patients have no evidence of streptococcal infection.
Pathology: the epidermis is generally normal, occasionally mild keratosis, collagen swells can be seen in the dermis, there is a clear gap between the collagen bundles or the formation of the window eye, the upper sweat squamous duct can be dilated, and there are large, small lymphocytes around the blood vessels. Mild infiltration, the appendage is generally normal, no scleroderma vascular occlusion changes, visible metaplasia, this metabolite disappeared by the treatment of hyaluronidase, indicating that the metachromatic is caused by hyaluronic acid.
Prevention
Hard swelling prevention
prevention:
1. Remove the inducement of the disease, pay attention to hygiene, strengthen physical exercise, improve your immunity, and prevent infection.
2, early diagnosis and early treatment, in the case of disease relief is not easy to give up treatment.
3, do a good job in maternal health, try to avoid premature birth, birth injury, suffocation. In the cold season, the newborn is warmed up and the temperature in the delivery room is adjusted. The degree is about 20 degrees, especially the warmth of premature and low birth weight children.
Complication
Sclerotherapy complications Complications acute renal failure cardiogenic shock
1. Circulatory failure in children with severe hypothermia, especially when the body temperature <30 ° C or hard swelling, often accompanied by obvious microcirculatory disorders, such as pale, blemishes, cold limbs, skin pattern, capillary filling time, Heart rate is fast and slow, heart sound is blunt and arrhythmia, severe heart failure myocardial damage and cardiogenic shock.
2. Acute renal failure This disease is often accompanied by urinary oliguria or even acute renal dysfunction without urine; severe renal failure occurs.
3. Pulmonary hemorrhage is the extreme performance of severe cases:
(1) Difficulty breathing and sudden increase in purpura, the symptoms are not relieved after oxygen administration.
(2) The wet rales in the lungs increase rapidly.
(3) Foamy blood is ejected from the nose and mouth or in the tracheal intubation.
(4) Blood gas shows that PaCO2 decreases PaCO2, and pulmonary hemorrhage is the most critical clinical symptom and main cause of death in this disease. It can die in a short time without first aid.
4.DIC: spontaneous bleeding of common skin and mucous membranes or injection of pinhole oozing, may be accompanied by shock and hemolysis.
Symptom
Symptoms of scleredema Common symptoms Skin involvement Low heat freckle pleural effusion Pericardial effusion
Symptoms include low fever, general fatigue, loss of appetite and insomnia, and early manifestations of generalized muscle pain. After the above prodromal symptoms, the skin may have progressive diffuse symmetry induration, often from the back of the neck and neck. Beginning on both sides, it is intractable waxy edema and spreads to the surrounding area. It can quickly affect the face, neck area, shoulders, upper arms, back and chest within a few days. The skin damage is non-depressed induration and loses normal skin. Texture and appearance, the surface is shiny and waxy, occasionally accompanied by a transient erythematous papule in the early stage of hard swelling. Although the lesion is usually located in the upper part of the trunk, it can sometimes extend to the abdominal wall, buttocks and thighs, and occasionally the calf can be affected. The hands and feet are generally not tired, no skin atrophy or hyperpigmentation, but often have ecchymosis-like skin lesions, chest and abdomen skin involvement sometimes cause breathing difficulties, pharyngeal involvement may cause difficulty swallowing, dysarthria, due to hard skin around the joints Swelling, it can cause a certain degree of dyskinesia. In addition to hard skin swelling, pericardial effusion, pleural effusion and joint effusion can still occur.
Examine
Examination of scleredema
(1) blood routine
There was no significant change in the total number of peripheral white blood cells. The total number of white blood cells and neutrophils in the combined infection may increase or decrease to varying degrees.
If the neutrophils are significantly increased or decreased, it indicates a poor prognosis.
(2) DIC screening test
The following six tests should be performed for patients with critical cirrhosis who are diagnosed with DIC:
1 platelet count: often progressive decline, about 2 / 3 children with platelet count <100 × 109 / L (100 000 / mm3).
2 prothrombin time: prolonged prothrombin time in severe cases, 20s in 4d after birth, 15s in age 5d and above.
3 part of the white clay soil thromboplastin time> 45s.
4 plasma thrombin time: normal neonatal value of 19 ~ 44s (elderly children 16.3s), compared with the same age of the control group > 3s has diagnostic significance.
5 fibrinogen <1.17g / L (117mg / dl), <1.16g / L (160mg / dl) has a reference value.
63P test (plasma protamine paracoagulation test): 65% of normal newborns have a 65% fibrinolytic activity, and may have fibrin degradation products (FDP), so the 3P test can be positive, and it is not normal after 24 hours. However, the DIC late 3P test can be turned negative.
(3) Blood gas analysis: Due to hypoxia and acidosis, blood pH decreased, PaO2 decreased, and PaCO2 increased.
(4) blood sugar: often reduced, may have creatinine, non-protein nitrogen increased.
(5) Ultra-micro-erythrocyte electrophoresis time measurement : due to the increase of blood viscosity, the red blood cell electrophoresis time is prolonged.
(6) ECG changes: Some cases may have ECG changes, manifested as QT prolongation, hypotension, T wave low or ST segment decreased.
Diagnosis
Diagnosis and diagnosis of scleredema
diagnosis
Diabetic patients with generalized central non-depression refractory edema, accompanied by erythematous lesions and pathological changes, can be diagnosed as this disease.
Scleredema is an uncommon disease that is usually benign. It is also a systemic rheumatism: it is characterized by a sudden onset of systemic symmetry and severe edema of the skin. The lesion characteristics were similar to those of the systemic scleroderma edema.
Differential diagnosis
1. Scleroderma: It is easy to be confused with scleredema in the early stage, but scleroderma has a history of Raynaud's episode and atrophic skin changes. Also. Scleredema rarely affects the hands and feet, and does not appear atrophy, hyperpigmentation and telangiectasia, combined with pathological changes can be identified.
2. Dermatomyositis: It is sometimes difficult to distinguish between onset and scleredema, but the former has elevated blood muscle enzymes, changes in electromyography and pathological changes of muscle inflammation, which can be differentiated from scleredema.
