1. Sleep scleroderma is the most common form of localized scleroderma, accounting for about 60%. According to the shape and scope of the lesion, it can be divided into oblique and universal. Sclerotinia sclerotiorum was originally a round, oval or irregular red or purple edema patch. After several weeks or months, it gradually expands, and the diameter is more than 1 ~ 10 cm. The central part is pale, pale yellow, ivory white or dirty grayish brown, with lavender or reddish halo around it. The surface is dry and smooth, with wax-like luster, leather-like hardness to the touch, hair shedding on it, no sweating, and sometimes accompanied by telangiectasia. After a slow process, after several years, the hardness decreased and gradually turned into white or light brown atrophic scars. It can occur anywhere, but it is more common in the abdomen and back of trunk, followed by limbs and face and neck. Permanent baldness can occur on the scalp, and it can cause deformity on the limbs.
Generalized scleroderma is rare, and the nature, occurrence and development of its lesions are similar to those of scleroderma maculata, but it is characterized by a large number of lesions, a wide range, up to dozens, and asymmetric distribution. The lesion eventually turned into a concave pale white atrophic plaque. It mainly occurs in the chest, abdomen and proximal limbs, and the face, neck, scalp, forearm and calf are relatively less involved. Although there is no organ involvement, patients may have joint pain, abdominal pain, migraine, neuralgia, mental disorder, emaciation, fatigue and so on. A few patients may turn into systemic sclerosis.
2. Linear scleroderma is more common in children and young people. Skin sclerosis is linear or banded distribution, which is easy to occur on forehead and involves face and head. The local skin is obviously atrophied and sunken, which can spread from the forehead to the top of the head, forming a band-shaped permanent alopecia spot, or it can spread down to the nose and mouth corner, resulting in atrophy and depression. The boundary is clear, like a sword, and 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 scleroderma, but the lesions are obviously sunken, and sometimes the muscles and bones under the lesions are also involved, which leads to decalcification, looseness and thinning of bones, and contracture can affect the function of nearby joints.
3. Scleroderma with unilateral atrophy This is a kind of segmental scleroderma that occurs on the face and is one of the reasons for unilateral atrophy. Taking the midline as the boundary, it often happens on one side of the face. The skin on the affected side is hardened, atrophied and sunken, which makes the face, eyes, nose and corners of the mouth askew. The fissure of the affected eye is narrowed, the alar can collapse and the affected side shrinks. This hard spot disease is also called Encoupdesabre.
4. This kind of skin damage is relatively rare, which can occur in the neck, chest, shoulders, back and so on. Usually the size of mung beans or soybeans, but occasionally nickels are larger and arranged in clusters or lines. The surface is smooth and shiny, slightly concave and hard in pearl or ivory color, and purple halo or surrounding pigmentation may appear for a long time.
The diagnosis is mainly based on the characteristic morphological changes of the skin, that is, the local skin is swollen, tight, hard, waxy, ivory, or accompanied by unilateral atrophy. Histopathological changes have reference value.
The clinical features and course of disease of patients with localized dermatoses and degenerative scleroderma are different from those of patients with diffuse dermatoses, as shown in Table 2. 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, severe lung, heart or kidney diseases are relatively rare. Prolonged course of disease may represent the slow manifestation of scleroderma, or it may indicate that localized scleroderma is a different disease.
Most localized scleroderma gradually develops into the clinical features of CREST syndrome, and the skin changes are limited to fingers (sclerosis changes). In addition to Raynaud's phenomenon, the first symptom of some patients is often heartburn or dysphagia. Severe gastrointestinal diseases with esophageal dysfunction and reflux are often persistent symptoms of such patients. Subcutaneous calcium deposition is characterized by local small masses, which often appear on fingers, forearms or other compression points. Especially in patients with crista syndrome, the number of dilated capillaries increases, which is common in face, mucosa and hands. The serious manifestations of localized scleroderma are pulmonary hypertension with or without pulmonary fibrosis and occlusive lesions of larger arteries, which are characterized by ischemia and amputation. Patients with localized scleroderma often have a syndrome that overlaps with another rheumatic disease.