1. Is skin hardening the first manifestation of scleroderma? Does every patient have symptoms of scleroderma? Scleroderma is a connective tissue disease characterized by fibrosis or sclerosis and eventual atrophy of the connective tissues of the skin and internal organs, which is an autoimmune disease. Clinically, the disease is divided into limited scleroderma and systemic scleroderma, the former only involves the skin, internal organs are generally not involved, and the prognosis is better; while the latter has extensive involvement of the skin and internal organs, if early treatment, standardized medication, most patients have a good prognosis, but a small number of patients due to the occurrence of pulmonary fibrosis, resulting in respiratory difficulties, pulmonary hypertension, and even pulmonary heart disease, the prognosis is worse, or even death. Restricted scleroderma can occur at any age and in any part of the body, and in the early stage, it can be manifested as hard spots, edematous plaques, dark red or purplish-red spots, or it can also appear as skin depressions and atrophic spots at the very beginning, while the texture is not hardened, such as banded or thread-like scleroderma. Deep-seated scleroderma presents as hard patches of skin in deeper locations. Therefore, not every patient’s lesions harden, but it is common for atrophy to eventually occur. Systemic scleroderma lesions are often manifested in three stages: ① edema stage: skin swelling, disappearance of dermatoglyphics, self-conscious tightness; ② sclerosis stage: the skin becomes hard, firm and shiny, grayish-yellow like wax, and the skin can not be pinched with the fingers; ③ atrophic stage: the skin is concave, atrophic, and varying degrees of thinning, and even tightly adhering to the bone, and the sclerosis of the skin in this stage is not obvious. 2.Generally in which parts of the first occurrence of sclerosis? Are all skin symptoms symmetrically distributed? Limited scleroderma can occur in any part of the body skin, single or multiple, but not necessarily symmetrically distributed; systemic scleroderma occurs symmetrically, often starting from the face and hands and feet, and gradually expanding to the forearms, legs and upper trunk, etc., but also the whole body skin hardening. 3.What is Raynaud’s phenomenon? Does Raynaud’s phenomenon occur in every patient? Raynaud’s phenomenon means that the skin of the fingers and toes appears pale, blue and purple, and finally flushed, which often occurs after nervousness and cold, mostly in the fingers, and the incidence rate of the toes is about 40%. It starts at the end of the fingers and gradually develops to the root of the fingers. It is characterized by pale skin on the fingers and toes, decreased skin temperature, tingling or numbness, dark red or purplish red skin after a few minutes, and finally red, swollen and painful skin. Raynaud’s phenomenon does not occur in limited scleroderma and only occurs in patients with systemic scleroderma. In addition, some other connective tissue diseases such as systemic lupus erythematosus, dermatomyositis, mixed connective tissue disease can also appear Raynaud’s phenomenon. 4.What are the different symptoms between limited and systemic scleroderma? Limited scleroderma only involves the skin, generally does not appear visceral involvement, the skin lesions can occur in any part of the body skin, can be single or multiple, asymmetric distribution, there is no systemic symptoms; systemic scleroderma in addition to skin damage, often accompanied by the involvement of internal organs, in particular, the lungs, esophagus, bones and joints, gastrointestinal and cardiac involvement, the lesions symmetrically occurring, often starting from the face and the hands and feet, and gradually expanding to the forearms, legs and upper trunk and other places. The skin lesions usually start from the face and hands and feet, and gradually extend to the forearms, legs and upper trunk, etc., or the skin of the whole body may become hard. Systemic scleroderma often has fever, arthralgia, myalgia, loss of appetite, Raynaud’s phenomenon and other systemic symptoms. 5. Can limited scleroderma develop into systemic scleroderma? Limited scleroderma and systemic scleroderma should belong to the spectrum of diseases, but limited scleroderma generally does not develop into systemic scleroderma. 6. How can scleroderma be distinguished from scleroderma, mixed connective tissue disease, sclerosing moss and other diseases? Scleroderma needs to be differentiated from scleroderma, mixed connective tissue disease and sclerosing moss. Scleroderma: mostly occurs after infection such as pharyngitis, mumps, etc., with sudden onset, often starting from the back of the neck and shoulders, and may develop to the face, front of the neck, scalp and chest and back, manifesting as swollen and hardened skin, which does not sink when pressed, and appears as a board or rubbery, with less involvement of the internal organs, and no Raynaud’s phenomenon. If there is difficulty in clinical diagnosis, histopathological examination will help in differential diagnosis. Mixed connective tissue disease: it is a disease with mixed manifestations of lupus erythematosus, dermatomyositis and scleroderma, which may also present with swelling and sclerosis of limbs, Raynaud’s phenomenon, and the manifestation of involvement of lungs, joints, esophagus, and other internal organs, which is not easy to be distinguished clinically from systemic scleroderma sometimes. However, due to the presence of high titers of anti-RNP antibodies in the patient’s serum and sensitivity to glucocorticoid therapy, it is now considered a separate disease. Mixed connective tissue disease may be considered if the clinical presentation meets the following criteria. ① Raynaud’s phenomenon or decreased esophageal peristalsis; ② severe myositis; ③ decreased carbon monoxide diffusion function <70%; ④ swelling and sclerosis of the fingers; ⑤ high titer positivity of serum anti-ENA antibody and anti-RNP antibody, and negative anti-Sm antibody; Sclerosing tundra: it can be seen in men and women of any age, and is predominantly found in the external genital and anal areas, with lesions of porcelain-white papules, plaques or plaques, and hardened and atrophied skin, and in severe cases Vulvar atrophy may occur. The histopathologic manifestations are similar to those of scleroderma, so it is currently believed that sclerosing moss and scleroderma may belong to the spectrum of diseases, only due to the difference in the degree of lesions, there is a difference in clinical and pathological manifestations with scleroderma. Scleroderma patient consultation guide 1. What information and photos are required for online consultation? What are the main points of disease description? Online consultation, please provide photos of skin lesions, preferably with distant and recent photos, in addition, please briefly describe the process of the disease, such as the site, time, symptoms, general condition and previous laboratory tests, treatment and medication, etc.; 2, scleroderma patients go to the hospital, what tests are usually done? Can it be done on the same day? How long does it take to get the test results? In most cases, limited scleroderma can be diagnosed by the characteristics of the skin lesions, and if necessary, a pathological biopsy can be done; in addition to biopsy, systemic scleroderma also needs to know the involvement of internal organs and systemic conditions, and autoantibodies, immune series, blood, urine, liver and kidney functions, chest X-ray, barium meal of the esophagus, pulmonary function, electrocardiogram, etc. can be done on the same day of the consultation. Laboratory tests can be done on the same day of the consultation, and labs can be obtained on the same day of most tests, but pathology biopsy may need to wait for 3-4 days. 3. If scleroderma is diagnosed, does it require hospitalization? Limited scleroderma is mostly treated in outpatient clinics, while systemic scleroderma is recommended to be hospitalized, and the treatment plan will be formulated according to the examination results. Generally, you can be discharged from the hospital in about 10 days, and go home to take the medication, and have regular outpatient checkups and follow-up visits. 4.Discharged from the hospital need to review? How often is the review? Can the review be done in the local hospital? Scleroderma is a chronic, intractable disease, patients need to be reviewed regularly, it is recommended once a month at the beginning stage, if the condition is well controlled, only a small dose of medication to maintain the treatment, can be reviewed once every three months. Routine checkups such as blood test, urine test, liver and kidney function, electrolytes, blood glucose, etc. can be performed in local hospitals.