Hyperextension of the skin

Ehlers-Danlos Syndrome or Ehlers-Danlos syndrome The disease is clinically manifested by three characteristics: excessive skin elasticity, weak skin and blood vessels, and large joint mobility. The disease is rare, with more males than females, and often has a family history. It is characterized by autosomal dominant inheritance and recessive inheritance. Hyperelastic skin syndrome is a rare congenital disease of connective tissue. Also called hyperdermatosis. The Danish dermatologist E. Elles reported in 1901 and the French dermatologist HA Danlow in 1908, so they were named after the two. It is characterized by increased fragility of the skin and blood vessels, which is easy to abrasion and atrophic scars; excessive elasticity of the skin, which can stretch the skin for a long time, several times more than normal, and quickly return to its original state after relaxation; excessive joint activity; bones, eyes, and internal organs also have Lesions. Occasionally, death can result from complications caused by visceral deformities. Clinically, it is characterized by excessive stretching of the skin and joints, easy damage to tissues, increased fragility and difficult healing of blood vessels, increased fragility of blood vessels, abnormalities of the eyes and abnormalities of internal organs. It is one of the major proteins of connective tissue, which is congenital metabolic abnormality. This disease is a hereditary disease, and its inheritance methods include autosomal dominant, autosomal recessive and sex-linked heredity. Family history is common. The pathogenesis is a disorder of collagen fiber metabolism. Except for areas of the skin that are secondary to changes due to trauma, most patients had no abnormalities in skin thickness, collagen fibers, and elastic fibers. Only in individual patients, collagen fibers are thin and not connected into bundles, and their skin is also thinner, and elastic fibers are relatively increased. The adventitia of the arterioles are defective and lack the support of surrounding connective tissue, so the blood vessels are vulnerable to damage. Symptoms occur at an early age, the patient's growth and development are retarded, and sometimes the onset is older. The skin is soft and elastic, it can be stretched very long, it can exceed the normal limit several times, and it can quickly return to its original position after relaxation. The skin on the neck, elbows and abdomen is saggy and wrinkled. The skin is fragile and fragile, prone to ecchymosis and hematoma, the latter can be transformed into small nodules, followed by calcification. Subcutaneous fat lobules tend to make weaker skin bulges appear pseudotumor-like. Wounds are not easy to heal, and the scars are thin and shiny, often accompanied by telangiectasias. Bones and joints are loose and overstretched, and joint hemorrhage and dislocation often occur. The knee can be flexed, and the spine is scoliosis. Other features include widening of the eye distance, myopia, lop ears, and flat feet. Congenital visceral deformities such as heart and lung malformations and gastrointestinal diverticulum are often complicated. According to clinical, genetic and biochemical defects, the disease is divided into 11 types. The diagnosis of typical cases is generally not difficult, but it must be distinguished from skin laxity. Skin sagging is characterized by loose and wrinkled skin, but does not increase in elasticity, and there is no excessive joint extension. There is no special treatment for this disease, and care should be taken to prevent trauma. Surgery should be performed with care, the wound is easy to crack and difficult to suture.

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