What is Scleroderma

Scleroderma is a connective tissue disease characterized by skin sclerosis or multi-organ fibrosis, which undergoes three stages: skin edema, sclerosis and atrophy. The former involves only the skin and presents with localized skin sclerosis; the latter is more common and is divided into limbic and diffuse forms, with “limbic” sclerosis starting in the extremities and “diffuse” sclerosis starting in the trunk, and may present with malignant hypertension. Systemic scleroderma involves extracutaneous organs, in order of incidence and severity, such as the lungs, heart, digestive tract, exocrine glands, kidneys, bone marrow and joints. Raynaud’s phenomenon (hands turning white and purple when exposed to cold) is characteristic of systemic scleroderma and occurs in almost all patients. There is also a specific type, CREST syndrome, which is characterized by calcium deposits on the skin, Raynaud’s phenomenon, abnormal esophageal motility, sclerosis of the fingers or toes, and capillary dilation. The disease has a serious and life-threatening impact on the quality of life of patients. Most of the disease has a prolonged course and only a few die within a short period of time, so it is important to be prepared for a long-term battle with the disease. Patients with systemic scleroderma must undergo a “systemic evaluation” upon admission to the hospital, i.e., an understanding of the status of the organs. The main treatment methods are as follows: I. Treatment for the skin Early treatment is the key. High-power UVA1 irradiation is effective for all types of scleroderma, especially limited, and can be cured after 3-6 months. Systemic scleroderma must be supplemented with drug therapy, commonly used are prednisone about 20mg/day and penicillamine in gradually increasing doses, long-term use of total peony glycosides as well as cumene tablets, ketotifen, etc. Second, for the treatment of various symptoms and organ damage 1, Raynaud’s phenomenon: warmth, smoking cessation is vital. Intravenous drip of salvia or prostaglandin and oral calcium channel blockers. 2. Interstitial pneumonia: prevent cold and flu, and choose the treatment plan according to the degree of lesion. 3, heart: except for severe cardiomyopathy can be effectively treated at present, such as pericarditis, pulmonary hypertension, etc. 4, digestive tract: keep the mouth clean, gargle or chew gum after eating; stand upright after meals, do not lie down within 30 minutes; inform the physician promptly when diarrhea, constipation and abdominal pain occur. 5, other, finger or toe ulcers to avoid touching, finger or toe continues to appear purple-black to promptly seek medical attention.