symptoms of progressive lipodystrophy, early symptoms and signs of progressive lipodystrophy
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Early symptoms: disappearance of fat tissue on the face or upper limbs
Late symptoms: changes in skin humidity, abnormal sweating, polyuria, impaired glucose tolerance, tachycardia, etc.
Related symptoms: loose skin, wrinkling, diabetes, body weight loss type, lower body weight loss type
Diagnosis of symptoms of progressive lipodystrophy
Progressive lipodystrophy (progressive lipodystrophy) is a rare autonomic nervous system disease characterized by adipose tissue metabolism disorder. The clinical and histological characteristics are slow and progressive bilaterally distributed basically symmetrical, well-defined, subcutaneous adipose tissue atrophy or Disappear, sometimes combined with limited adipose tissue hyperplasia and hypertrophy.
Due to the different scope of lipodystrophy, it can be divided into localized lipodystrophy (Simons disease or head and chest lipodystrophy) and systemic lipodystrophy (Seip-Laurence syndrome).
1. Most patients onset around the age of 5 to 10, women are more common, onset and progress are slower. At the beginning of the disease, the fat tissue of the face or upper limbs disappeared in most patients, and then it expanded downward and affected the buttocks and thighs, showing a roughly symmetrical distribution. The course of the disease lasts for 2 to 6 years and can stop by itself. The patient’s face is characterized by recessed cheeks and temples on both sides, loose skin, loss of normal elasticity, and loss of fat around the cheeks and orbits, giving the patient a special appearance. In some patients, there may be obvious subcutaneous tissue hyperplasia and hypertrophy in the buttocks and hips, but the hands and feet are often unaffected.
2. The patient can manifest as the coexistence of adipose tissue disappearance, special obesity and normal adipose tissue, combining the basic characteristics of the disease in different ways. According to different combinations, it can be expressed as the following types:
①The upper body is normal, and the lower body is obese;
②The upper body is thin and the lower body is obese;
③Simple upper body wasting type;
④The upper body obesity type;
⑤ Lower body weight loss type;
⑥ Body weight loss type;
⑦Half body obesity type.
3. Patients may be combined with changes in skin moisture, abnormal sweating, polyuria, impaired glucose tolerance, tachycardia, unstable vascular movement, vascular headache, abdominal pain, vomiting, skin and nail nutritional disorders and other autonomic dysfunction manifestations, individual Cases can be combined with endocrine dysfunction, such as dysplasia of reproductive organs, abnormal thyroid function, acromegaly and menstrual disorders. Symptoms generally stabilize within 5 to 10 years after the onset of disease.
4. The patient's muscles, bones, hair, breast and sweat glands are all normal, without muscle dysfunction, the physical strength of most patients is not affected, and the physical and mental development in the advanced stage of the disease is not affected. It has recently been reported that Hodgkin's disease and scleroderma can be complicated.
5. Newborns or infants and young children often have congenital systemic and multi-organ diseases, in addition to involving the head, face, neck, trunk and limbs, including the subcutaneous and visceral adipose tissues, it can also be accompanied by high Blood lipids, diabetes, hepatosplenomegaly, skin pigmentation, heart and muscle hypertrophy, etc.
According to the disappearance of subcutaneous fat tissue, normal muscle and bone quality, biopsy of fat tissue disappearance, subcutaneous fat loss, increase and normality, the three conditions can be combined in different ways to confirm the diagnosis.
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