Overview.
Segmental hyaline vasculitis, also known as Milian white atrophy, glaucomatous vasculitis disease, glaucomatous vasculitis, glaucomatous vasculitis, and painful purpuric ulcers of the lower extremities of the reticular type, is a chronic relapsing segmental vasculopathy of the small dermal blood vessels (thrombotic disease). This disease is in the two lower limbs on the basis of small vascular lesions, polymorphic skin damage occurs after healing, leaving atrophic scarring vasculitis disease. It occurs mostly in young and middle-aged women. In most cases, the disease worsens in the summer and rarely decreases in the winter.
Causes
1. Causes
The most common cause is chronic venous insufficiency. It can also be combined with nevus erythematosus, thalassemia minor, cryoglobulinemia, SLE and scleroderma.
2. Pathogenesis
It is different from leukocytoclastic vasculitis because there is little neutrophil infiltration and nuclear dust in the histologic lesions.
Proliferation of endothelial cells in dermal capillaries, proliferation of endothelial cells in some of the fine veins in the superficial intervals of subcutaneous adipose tissue, thickening of the wall due to eosinophilic deposition, hyaline thrombosis in the lumen, perivascular erythrocyte leakage, predominantly lymphocyte infiltration with occasional neutrophils and nuclear dust, and mild hypertrophy of epidermal spiculocytes, or vacuolar degeneration and necrosis.
Symptoms
The damage of this disease is firstly seen in the lower part of both sides of the lower leg, especially the inner and outer ankle and its surrounding, and then slowly upward to the upper part of the knee joint, and down to the dorsum of the foot, the end of the toes and the soles of the feet, and the primary damage is a pinhead-sized bright red petechiae, which is distributed in a ring shape or densely packed such as a finger-sized patch, and the central part of the petechiae is gradually turning into a dark purplish red petechiae, and then it further becomes a black scab, which sometimes is thicker, and is surrounded by a greyish-white blister with a flaccid wall, and the scabs are under the mungbean to soybean size and shape. Under the scab is a green bean to soybean sized, irregularly shaped ulcer with irregular edges. The pain is most severe when bruised and can be convulsive. Sometimes only light red limited elevation pain and pressure pain is also more significant. Slow healing of the ulcer, leaving a yellowish atrophic scar, or only yellowish pigmented spots at the same time in the interspace scattered white atrophic scar ulcers in the lower calf and the inner and outer ankles, mainly the course of the disease for the chronic, recurring, the duration of the disease, the longest more than 5 years. About half of the patients in the calf 1 ~ 3 years after the onset of the lower thighs in the lower part of the two thighs occur reticular cyanotic spots, and winter or cold when the appearance, warm and summer disappeared, but does not develop into an organic lesion. The reticular bruising does not disappear after the calf damage has healed.
Examination
Histopathological examination may show epidermal atrophy, V-shaped necrotic areas in the epidermis and upper dermis, thickening of the vessel wall, deposition of fibrin-like material in the wall and lumen, partial or complete occlusion of the lumen, spillage of erythrocytes, and periportal infiltration with lymphocytes and histiocytes.
Diagnosis
Diagnosis is mainly based on clinical manifestations and histopathologic examination.
Differential diagnosis
In skin lesions on the ankle, primary or secondary hypercoagulable states should first be excluded, especially diseases associated with antiphospholipid antibody syndrome, fibrinolytic abnormalities of viral hepatitis C, etc. and venous and arterial peripheral vascular diseases. Skin lesions occurring in other parts of the body should be differentiated from DLE, sclerosing atrophic moss or malignant atrophic papulosis.
Treatment
1. Patients should avoid localized bruising, abrasions, contact with known allergens, or wearing uncomfortable shoes.
2. Intradermal injections of lidocaine and triamcinolone acetonide are effective in relieving pain, after which compression therapy may be used.
3. Treatment with antiplatelet agents such as aspirin, dipyridamole, and beclomethasone can be effective.
4. For the hypofibrinolytic nature of the disease, low molecular dextran can be used.
5. Secondary infection of ulcers should be treated systematically with antibiotics, and skin grafting is feasible after infection control. Photochemotherapy has been reported to be effective.