Overview of localized scleroderma
Localized scleroderma, also known as scleroderma, is a kind of skin disease with local swelling and gradual hardening and atrophy. It is common in scalp, forehead, waist, abdomen and limbs. The skin lesions are reddish and slightly edematous in size, single or multiple. Later, it gradually hardened and became pale yellow or yellowish white. The surface is smooth and shiny like wax, the center is slightly concave, the hair at the lesion falls off, sweating is reduced, and the surrounding capillaries are dilated, purple or pigmented. Advanced skin atrophy, hypopigmentation. Skin lesions have different shapes, which can be divided into four types: patch, strip, drip and generalized, of which patch is the most common. The disease generally has no conscious symptoms, some of which may have mild itching or tingling, and gradually become dull without obvious systemic symptoms. Localized scleroderma generally does not invade internal organs.
Patchy scleroderma
1. Scleroderma: The skin lesions are reddish or purplish edematous lumps (plaques) at the initial stage (while the central part is shiny and hardened), slightly higher than the skin surface, lasting for a long time, and gradually expanding after several weeks or months. After a few months, the red color gradually turns pale, showing yellowish or ivory white, and the periphery is often surrounded by lavender or reddish halo (the unique inflammatory blush on the edge is called lilae wheel). The surface is dry and smooth, not sweating locally, the hair gradually disappears, showing wax-like luster, leather-like hardness to the touch, and sometimes accompanied by telangiectasia. After a slow process, after several years, the hardness decreases, local thinning and atrophy, leaving a slight pigmentation (progressive pigmentation or loss). Local skin depression. It's good. It can happen anywhere, but the trunk is the most common. This shape is the most common in the limited type, accounting for about 60%.
2. Generalized scleroderma: rare, mostly scleredema plaques, with varying degrees of hypopigmentation or pigmentation, usually occurring in the chest and abdomen, proximal limbs and buttocks, but the face, neck, scalp, forearm and calf can also be involved. There is no obvious systemic damage, and it usually stops developing after 3 ~ 5 years. Its occurrence and development are similar to scleroderma maculatum, but it is characterized by a large number of skin lesions, a large area of skin sclerosis, wide distribution and no systematic damage. It can often be combined with joint pain, neuralgia, abdominal pain, migraine and mental disorder. RA reaction and anti-ENA antibody are positive, and localized permanent alopecia often occurs in hair. A few patients can turn into systemic scleroderma.
Scleroderma banded
Zonal scleroderma: more common in children and adolescents, it is often distributed in a linear or banded shape along the extended side of one limb. Similar to flake injury, but the skin injury has obvious depression. At the beginning, the skin was mostly in a slightly concave and atrophied strip or linear shape, and the skin adhered to subcutaneous fat and myofascia, gradually forming linear skin sclerosis, limited activity or causing limb twins and bone development disorders. Scalp or forehead can also occur, the process is similar to flake, but the skin lesions are obviously sunken, sometimes knife-shaped (scleroderma sword). Sword scleroderma: a kind of banded scleroderma, which mostly extends to the scalp near the middle of the forehead in a knife shape, showing milky sclerosis and obvious local depression. Skin, subcutaneous soft tissue and bone gradually atrophy and sag, accompanied by depigmentation. Sometimes decalcification, looseness, absorption thinning and hair loss can occur in muscles and even bones under skin lesions, and in severe cases, ipsilateral atrophy and ipsilateral tongue atrophy can be formed (with unilateral atrophic scleroderma of the face).
Scleroderma with unilateral facial atrophy: a special type of banded scleroderma, with facial atrophy and unilateral atrophy, without conscious symptoms. If there is unilateral facial atrophy with ipsilateral tongue atrophy, it is called Parry-Rhomberg syndrome), a few cases may be accompanied by spina bifida. General unconscious symptoms, occasionally mild itching, pain or decreased consciousness.
Drip scleroderma
The damaged soybean is about the size, dense and non-fused, white or ivory in color, smooth in surface, with pigmentation around it, which shrinks after a long time. It occurs in chest, neck, shoulders and other parts.
Generalized scleroderma
Patchy, banded and dribbling scleroderma lesions can coexist partially or completely, distributed in all parts of the body, with many lesions, but rare in the face, and often tend to merge. Often complicated with joint pain, abdominal pain, neuralgia, migraine and mental disorders, and occasionally can turn into systemic scleroderma.
Differential diagnosis between localized scleroderma and other diseases
1. Macular atrophy (progressive idiopathic skin atrophy): the early damage is round or irregular reddish patches of different sizes, and then gradually shrinks, showing skin color or bluish white, slightly concave or convex, with wrinkles on the surface and difficult to touch. Different from scleroderma, it can be distinguished.
2. Sclerosing atrophic moss: at the initial stage of the disease, it is reddish papules from the tip of the needle to the flat head of peas, and then it is milky white or mother-of-pearl, with a solid texture and gradually flattening, sometimes lower than the normal skin surface. There are tiny black spots of keratin embolism on the surface of the papule, and in addition, there may be different white patches with mild hardening at the beginning. In the later stage, papules and plaques flatten or even sink, and the skin lesions may have a parchment-like appearance, usually with hairy skin.
Frontal banded scleroderma (scleroderma sword)
Zonal scleroderma, in which skin lesions are distributed in strips along ribs or one limb, or occur in a knife-shaped or concave strip on forehead, and eyebrows fall off. Hair on the forehead can extend from the scalp to the front and even cause facial atrophy. Scleroderma banded often involves superficial and deep subcutaneous fat, muscle, fascia and even bones, which makes bones decalcified, loose and absorbed, resulting in serious deformity, especially in children during their growth and development. Lesions in elbow, wrist and finger joints can restrict joint activity or make limbs bow contracture deformed, which is more common in children and adolescents. Hair on the scalp: at the initial stage, the hair on the scalp is hard (a few are not hard), the skin is tight and bright (some patients only shrink), it is difficult to pick it up by hand, and it is flushed locally, generally without discomfort. With the development of the disease, local atrophy appears, and the lesion area expands, which can appear in all parts of the scalp and develop downwards, eventually leading to unilateral facial atrophy; It can also develop to the neck and other parts of the body.
Forehead hair: in the early stage, the affected part is strip-shaped, which can be moss-like or vitiligo-like, but it is not hard, the surface is rough, bright and atrophied; Or the skin is hard, shiny and pigmented. The lesion area expands with the development of the disease, and the degree of atrophy deepens.
The disease generally has no conscious symptoms, some of which may have mild itching or tingling, and gradually become dull without obvious systemic symptoms. Localized scleroderma generally does not invade internal organs. No visceral damage and serological changes were found.