Scleredema

Introduction 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.

Cause

The newborn's body surface area is relatively large, the skin is thin and tender, and the blood vessels are rich. It is easy to dissipate the complex color fat. It is a unique tissue in the newborn. Its metabolism is the main source of energy when the newborn is in urgent need of heat production in a cold environment. The source of energy is white fat, such as the ambient temperature in children is too low, too much heat, brown fat is easy to deplete body temperature will drop. The body temperature will not rise when the newborn is seriously infected. In these cases, the subcutaneous fat is easy to coagulate and harden, and at the low temperature, the surrounding capillaries are dilated, the permeability is increased, and the edema is prone to hard swelling. The study of the effect of hypothermia on the human body is still deepening. When the body temperature is low, the peripheral circulation resistance decreases, the blood stasis, the blood circulation of the tissue hypoxia center decreases, the heart rate slows down, and the urine volume decreases during the rewarming process, such as urine. The amount does not increase, may cause heart failure and even pulmonary edema and pulmonary hemorrhage; 2 slow breathing at low body temperature, sometimes apnea, prone to respiratory acidosis and due to insufficient nutrient intake, resulting in metabolic acidosis, so heavy Hard swollen acidosis is also heavier; 3 poor glucose metabolism at low body temperature may cause hyperglycemia at the beginning of the disease, but due to increased sugar consumption, hypoglycemia occurs; 4 hematocrit and blood viscosity increase at low body temperature Reduced, heparin-like substances are also reduced. Various causes can cause disseminated intravascular coagulation (DIC) induced by coagulopathy. DIC is more likely to occur due to shock during severe infection.

an examination

Related inspection

Body temperature measurement of blood glucose

1. The medical history is in the cold season, the ambient temperature is too low or there is a history of improper warmth; the history of serious infection; premature or full-term small samples; insufficient intake due to asphyxia, birth injury, etc. or low energy supply.

2. Clinical manifestations of poor early lactation, low crying, low response, body temperature <35'E, severe <30'E, temperature-anal temperature difference from positive to negative, infection or summer incidence A hypothermia occurs. Hard swelling is symmetrical, followed by lower limbs, hips, cheeks, upper limbs, back, abdomen, chest, etc. In severe cases, the limbs are stiff and inactive. Multiple organ function damage.

Differential diagnosis

Differential diagnosis: This disease sometimes has edema, which should be differentiated from neonatal edema. At the same time, it should be differentiated from neonatal subcutaneous gangrene and neonatal subcutaneous fat necrosis.

1. Clinical manifestations: This disease often occurs in the cold season. It is more common in babies shortly after birth or within 1 week. There are many cases where the ambient temperature is low and the warmth is not enough. Because of premature birth, infection and other factors, it is also seen in summer children. The body temperature drops to 31 ~ 35 ° C, or even about 26 ° C, can be used in China's self-made low thermometer (30 ~ 40 ° C) to detect low crying or not crying, can not suck, the body spontaneous movement less skin first dark red, then turned to Dark red, severely pale or bruised limbs and trunk are cold, and the veins are weak and difficult to reach. The skin and subcutaneous tissue first become hard after edema, and the severe ones are like hard rubber. Hard swelling occurs first in the cheeks and shoulders of the calves, and later on the outside of the thighs, the upper limbs of the buttocks are also affected, and even the whole body is involved. Difficulty breathing due to hard and swollen chest and abdomen can not open mouth due to hard swelling of the cheeks. The child's heart sound is low and blunt, heart rate slows down, low urine, and even no urine. Later, hemorrhagic fluid in the nose and nose will cause pulmonary hemorrhage and die.

2. Diagnosis of sclered lesions: There is no consensus. There are also light, moderate and severe according to the general condition of the hard swelling range, body temperature, and the presence or absence of shock pulmonary hemorrhage.

3. Diagnosis of skin hard swelling range: according to the size of skin hard swelling, light, moderate and severe mild: hard swelling range is less than 30%; moderate: hard swelling range is 30% to 50%; severe: hard swelling range More than 50%.

4. Hard swollen subcutaneous fat toughness diagnosis index: I degree: subcutaneous fat is slightly hard, skin color is slightly red; II degree: edema is more obvious, subcutaneous fat elasticity basically disappears skin color is slightly dark red; III degree: edema is obvious, The subcutaneous fat elasticity disappears, and the rubber-like hard skin is dark red.

6 The diagnostic criteria for critical scleredema are based on the Trial Program for Critical Case Scoring Method proposed by the Office of Children's First Aid Project of the Ministry of Health of the Ministry of Health.

(1) The temperature of the anus is more than 2 degrees above 30 °C, regardless of the size.

(2) The temperature of the anus is more than 2 degrees above 33 °C, and the range is more than 60%. Anyone who meets one of the above two conditions can be diagnosed as critically ill. This disease occurs more than 7 to 10 days after birth, body temperature does not rise, below 35 ° C, severe below 30 ° C core temperature (anal temperature) may be lower than body surface temperature ( temperature), skin and subcutaneous tissue appears hard The skin is light red or dark red. The severe circulation may be pale gray or blue-purple. Hard swelling first appears in the lower limbs, buttocks and lower abdomen, and then to the upper limbs and the whole body. Sometimes only hard and not swollen, the skin color is pale, like rubber, the scope of the limitation only affects the thigh and buttocks. This situation often occurs in the hard swelling caused by infectious diseases. Severe scleredema can cause shock pulmonary hemorrhage and DIC.

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