localized scleroderma
Introduction
Introduction to localized scleroderma As the name implies, scleroderma is a disease in which the skin becomes hard. The general classification restricts the lesion to the skin. The viscera is not affected by the localized scleroderma (localizedscleroderma), located at one end of the spectrum, systemic sclerosis ( Systemicsclerosis) diffuse scleroderma (diffusescleroderma), extensive skin lesions with multiple organ involvement at the other end of the spectrum. Between the two poles, there are some intermediate types, such as generalized scleroderma in localization, acral scleroderma in systemic, CREST syndrome, etc., and such classification is listed in Table 1. The simple invasion of the skin is limited, the general prognosis is good, the 10-year survival rate is 70%. basic knowledge The proportion of illness: 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: congenital multiple joint contracture
Cause
The cause of localized scleroderma
(1) Causes of the disease
The exact cause of the disease is unclear and may be related to infection, genetics, and environmental factors.
(two) pathogenesis
The pathogenesis of this disease is still not very clear. In general, there are vascular injury theory, immunological damage theory, and fibrous abnormal hyperplasia theory. See Systemic scleroderma for details.
Prevention
Localized scleroderma prevention
1. Remove the inducement of the disease, pay attention to hygiene, strengthen physical exercise to improve immunity and prevent infection.
2. Early diagnosis and early treatment, do not give up treatment easily when the disease is relieved.
Complication
Localized scleroderma complications Complications congenital multiple joint contracture
Hard spot disease occurs in the scalp. Permanent alopecia can occur. It can cause deformity on the extremities. Skeletal involvement of scleroderma can cause joint contracture. Hard spot disease with facial atrophy can make face, eye, nose and mouth. Tilt and nose collapse.
Symptom
Localized symptoms of scleroderma Common symptoms Skin sclerosing Hair loss Permanent alopecia Neuralgia Weak abdominal pain Edema
The skin manifestations and histopathological changes of localized scleroderma are similar to those of systemic scleroderma, but no visceral involvement.
1. Morphosis (morphea)
Hard spot disease is the most common in localized scleroderma, accounting for about 60%. According to the morphology and extent of the lesion, it is divided into plague morphea and generalized morphea, plaque plaque. From the beginning, it is a round, elliptical or irregular reddish or purple-red edematous flaky lesion, which gradually enlarges after several weeks or months, and the diameter can reach 1 to 10 cm or more, and the central part turns light. It is light yellow ivory white or stained grayish brown. It is surrounded by pale purple or reddish halo. The surface is dry and smooth, with waxy luster. It has a leather-like hardness. It has hair loss, no sweating, and sometimes accompanied by capillaries. Dilation, after a slow, several years later, the hardness is reduced, gradually becoming a white or light brown atrophic scar, which can occur in any part, but in the abdomen of the trunk, the back is more common, followed by the limbs and the neck and neck, when it occurs in the scalp Permanent alopecia can occur, which can cause deformities at the extremities.
Generalized scleroderma is rare, and the nature of its lesions occurs and develops like plaque plaque, but it is characterized by a large number of lesions, extensive lesions, up to tens of pieces, asymmetric distribution, and lesions eventually become Depressed pale white atrophic patch, occurs in the chest and abdomen and proximal extremities, face, neck, scalp, forearm, calf involvement is relatively small, although no organ involvement, but patients may have joint pain, abdominal pain, migraine , neuralgia and mental disorders, weight loss, fatigue, etc., a small number of patients can be converted to systemic sclerosis.
2. Sessile scleroderma (linear scleroderma)
Banded scleroderma is more common in children and young people. The skin is hardened in a line or band shape. It occurs in the forehead and can affect the face and head. The local skin is obviously atrophied, and the depression can be offset from the midline to the side. The part spreads up to the top of the head, and a band-shaped permanent alopecia spot occurs, which spreads down to the nose and mouth. The atrophy, depression, and clear boundary, such as sword-cutting, finally the skin is thin and atrophy, like sticking to the bone surface. The damage may also be distributed along the limbs or intercostals, which may vary from single to several, and the morphological characteristics are similar to those of the hard spot disease, but the lesions are obviously sunken, and sometimes the muscles and bones under the skin lesions are also affected. Decalcification, loosening, and thinning of the bone can occur, and nearby joints can affect function due to contracture.
3. Hard spot disease with facial fascia (morphea with facial hemiatrophy)
This is a kind of segmental morphea disease that occurs on the face. It is one of the causes of eccentric atrophy. It is demarcated by the midline and often occurs on one side of the face. The skin is hardened, atrophied, and sunken. Eyes, nose and mouth are skewed, the affected eye is narrowed, the nose can collapse, and the affected side is atrophied. This kind of hard spot disease is also called En coup de sabre.
4. Drop scleroderma (guttate morphea)
This type of skin lesions are relatively rare, can occur in the neck, chest, shoulders, back, etc., usually damaged by the size of mung bean or soybean, but occasionally 5 cents coin large, clustered or lined, is a smooth surface Shining, a slightly concave and hard small spot in mother-of-pearl or ivory, surrounded by a purple-red halo or pigmentation, which can shrink over time.
The diagnosis is mainly based on the characteristic morphological changes of the skin, that is, the swelling of the local skin, tightening, hardening, waxy luster, ivory color change, or facial atrophy, and histopathological changes have reference value.
The clinical features of patients with localized cutaneous scleroderma are different from those of diffuse type. These patients often have Raynaud's phenomenon for many years (sometimes more than 10 years) before the appearance of other signs of scleroderma, although they may be accompanied by organ damage. However, severe lungs, relatively few heart or kidney lesions, this extended course of disease may represent a slow expression of scleroderma, and may also indicate that localized scleroderma is another different disease.
Examine
Localized scleroderma examination
The measured time value of the affected or unaffected skin was significantly longer than normal, up to 5 to 12 times normal.
1. Histopathological examination
Fibrosis and microvascular occlusion are characteristic pathological changes in all affected tissues and organs of patients with sclerosing disease.
2. Skin pathology examination
In the early stage, dermal interstitial edema, collagen fiber bundle swelling, lymphocyte infiltration around the collagen fibers and small blood vessels in the dermis, mainly T cells, late, dermal and subcutaneous tissue collagen fibers proliferated, dermis thickened, collagen swelling, Fibrosis, destruction of elastic fibers, thickening of blood vessel walls, narrowing of the lumen, and even occlusion. Later, the epidermis, skin appendages and sebaceous glands atrophy, sweat glands are reduced, and calcium deposits in the deep and subcutaneous tissues of the dermis.
Diagnosis
Diagnosis and diagnosis of localized scleroderma
Diagnostic criteria
Most localized scleroderma develops into the clinical features of CREST syndrome. Skin changes are limited to fingers (hardening changes). In addition to Raynaud's phenomenon, patients with localized symptoms often have heartburn or difficulty swallowing, severe gastrointestinal problems. Pathological dysfunction with esophageal dysfunction and reflux is often a persistent symptom in these patients. Subcutaneous calcareous deposits are localized small lumps that often appear on the fingers, forearms or other points of compression, especially in patients with CREST syndrome. Increased number of expanded capillaries, common in the face, mucous membranes and hands, severe manifestations of localized scleroderma are pulmonary hypertension and large arterial occlusive lesions with or without pulmonary fibrosis, manifested as ischemia and Amputation surgery requiring amputation and coexistence with another rheumatic disease occurs in patients with localized scleroderma.
Differential diagnosis
1. macular atrophy
Occurs in the trunk, the round or oval red patches in the early stage of skin lesions are about 1cm, and then gradually shrink, the skin lesions can be single or multiple clusters, the surface skin can be normal or blue-white, obviously concave, but The patient is not hard to touch, the patient has no symptoms, the young is more common, and the cause is unknown.
2. progressive idiopathic atrophoda (progressive idiopathic atrophoderma)
The disease occurs in the trunk of the young and middle-aged, and can also be found in the extremities. The cause is unknown. The early lesions are irregular patches with unclear boundaries, ranging from 1 to 10 cm in diameter. The surface is gray or tan, and the skin is thin and thin. It is soft, gradually shrinking, slightly depressed, and can be hardened in the center of the atrophic plaque. The histopathological changes are deeper collagen fibers in the dermis and the glassy degeneration. This histological change and the skin's first atrophy and hardening characteristics are easy and hard. Identification of spot disease.
3. Atrophic sclerosing moss (lichen sclerosus et atrophicus)
Also known as white lichen (lichen albus) or white spot disease, occurs in the vulva, but also in the neck, chest, breast and back, the skin lesions are smooth, slightly wrinkled gray or ivory white patches There are purple-red halos around, some plaques are scattered in white spots, may be accompanied by mild sclerosis, histopathological changes to epidermal atrophy, dermal superficial homogenization changes, inflammatory cell infiltration.
