Etiology of localized scleroderma
(A) the cause of the disease
The exact cause of the disease is not clear, which may be related to infection, heredity and environmental factors.
(2) Pathogenesis
The pathogenesis of this disease is not very clear. Generally speaking, there are vascular injury theory, immune injury theory and fibrous dysplasia theory. See systemic scleroderma for details.
Symptoms of localized scleroderma
Constant 1. Sclerotinia sclerotiorum
Scleroderma is the most common localized scleroderma, accounting for about 60%. According to the shape and scope of the lesion, it can be divided into plague sleeping and generalized scleroderma. Plaque scleroderma is a round, oval or irregular reddish or purplish edema, which gradually expands after several weeks or months. The diameter can reach more than1~10cm. The central part is pale, pale yellow, ivory white or dirty grayish brown, and the periphery is faintly lavender or reddish. The surface is dry and smooth, with waxy luster and leather hardness. The hair on it falls off without sweating, sometimes accompanied by telangiectasia. After several years, the hardness decreases and gradually turns white or light brown and shrinks.
Generalized scleroderma is rare, and its skin lesions are similar in nature to plaque scleroderma, but it is characterized by a large number of skin lesions, which can reach dozens and are asymmetrical in distribution. The lesions eventually turned into concave pale white atrophic plaques, which were mostly found in the chest, abdomen and proximal limbs, and the face, neck, scalp, forearm and calf were relatively less involved. Although there is no organ involvement, patients may have joint pain and abdominal pain.
2. Linear scleroderma
Scleroderma banded is more common in children and young people. Skin sclerosis is linear or banded distribution, which is easy to occur on the forehead and can involve the face and head. The local skin is obviously atrophied and sunken, which can spread from the forehead to the center line to the top of the head, and there is a band-shaped permanent alopecia spot, which can spread down to the nose and mouth corner, shrink and sag, and the boundary is clear, such as cutting with a knife. Finally, the skin becomes thinner and shriveled, as if sticking to the bone surface. Lesions can also be distributed in strips along limbs or ribs, ranging from one to several. The morphological characteristics and process are similar to those of scleroderma, but the lesions are obviously sunken. Sometimes the muscles and bones under the lesion are also involved, and the bones are decalcified, loose and thin. Contraction can affect the function of nearby joints.
3. Sclerotinia sclerotiorum with facial hemiatrophy.
This is a kind of segmental scleroderma of the face, which is one of the reasons for unilateral atrophy. Delineated by the midline, it often occurs on one side of the face. The skin of the affected side is hardened, atrophied and sunken, which makes the face, eyes, nose and corners of the mouth askew, the cleft lip of the affected side becomes narrower, and the alar can collapse, resulting in atrophy of the affected side. This scleroderma is also called a fatal blow.
4. Dripping scleroderma
This type of skin lesions is relatively rare, which can occur in the neck, chest, shoulders, back and so on. It is generally the size of mung beans or soybeans, and occasionally it is as big as a nickel coin, showing clusters or lines. It is smooth and glossy, with mother-of-pearl or ivory-colored micro-concave hard spots, surrounded by purple halo or pigmentation, which can shrink for a long time.
The diagnosis is mainly based on the characteristic morphological changes of the skin, that is, local skin swelling, tightness, hardening, waxy, milky white, or accompanied by unilateral atrophy. Histopathological changes have reference value.
The clinical features and course of disease of localized dermatoses and degenerative scleroderma are different from those of diffuse dermatoses. These patients often have Raynaud's syndrome for many years (sometimes more than 10 years) before other signs of scleroderma appear. Although they may be accompanied by organ damage, serious lung, heart or kidney diseases are relatively rare. This prolonged course of disease may represent a slow manifestation of scleroderma, or it may mean that localized scleroderma is a different disease.
Treatment of localized scleroderma by western medicine
Patients with localized scleroderma can take large doses of vitamin E, compound phosphate tablets or trihexyphenidyl orally. You can also take traditional Chinese medicines for promoting blood circulation, removing blood stasis, dredging menstruation and activating collaterals, such as Danshen tablets or Fuchun tablets. Skin lesions can be treated with topical corticosteroids, or local skin lesions can be injected with triamcinolone acetonide once a week/kloc-0, 4-6 weeks 1 course of treatment. If skin atrophy occurs, the injection will be stopped. Intralesional injection of 150mg hyaluronidase every day also has a certain effect. Physical therapy can also be used, such as hydrotherapy, wax therapy, audio electrotherapy, massage, massage and so on. For patients with joint contracture, surgical treatment is feasible when necessary, which can relieve contracture and restore activity function.
prognosis
The cause of localized scleroderma is not visceral involvement, so the prognosis is good. After treatment, the hard lumps on the patient's skin can fade or relieve themselves, leaving pigmentation or atrophic scars. The prognosis of patients mainly depends on the degree of involvement of internal organs, and the mortality rate of scleroderma is higher than that of the general population. The one-year survival rate of diffuse type 10 was 50%, and the prognosis was poor, while that of localized type was 70%. The main cause of death of the former is scleroderma-related kidney, lung and heart diseases, while the main cause of death of the latter is pulmonary hypertension. Cardiopulmonary diseases are the main cause of death in scleroderma. The older the onset, the worse the prognosis. In short, the survival rate depends on early diagnosis and early active treatment.
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