Lipid progressive necrosis
Introduction
Introduction to lipid progressive necrosis Necrobiosis Lipoidica, also known as progressive lipid necrosis of diabetic lipids, is characterized by large scleroderma-like plaques, often associated with diabetes. The disease is a large piece of scleroderma-like damage that occurs before the sputum. It is more common in women, and some patients are associated with diabetes. In 1929, Oppenheim first reported one case, followed by Urbach to report the second case and named progressive diabetic necrosis. basic knowledge The proportion of illness: 0.0006%--0.0008% Susceptible people: no specific population Mode of infection: non-infectious complication:
Cause
Fatty progressive necrosis
(1) Causes of the disease
The cause is still unclear. There are two possibilities. The patient has 2/3~3/4 of diabetes, so one is considered to be related to diabetes, accompanied by diabetic microangiopathy, resulting in glycoprotein in the small blood vessel wall. Sedative, gradually causing vascular occlusion and tissue necrosis, another is considered to be unrelated to diabetes, only 0.3% of all patients with diabetes, and the course of the disease is not related to the severity of diabetes, the duration of disease and the controlled condition .
(two) pathogenesis
The mechanism of progressive lipid necrosis has not been fully elucidated. It has been found by direct immunofluorescence that IgM, IgA, C3 and fibrinogen deposits in the blood vessel wall of affected lesions, and fibrinogen deposition in the epidermal dermal junction. In the progressive necrotic area, fibrinogen is massively deposited, suggesting that the disease belongs to immune complex vasculitis. In addition, the level of autoantibodies against various cytoskeletal components in serum is significantly higher than that of normal people, including anti-keratin. Proteins, troponin, myosin, etc., the cause and significance of which are still unclear, some analysts believe that these autoantibodies may be related to the formation of immune complexes.
Prevention
Lipid progressive necrosis prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Progressive lipid necrosis Complication
Generally no complications.
Symptom
Fatty progressive necrosis symptoms common symptoms nodular hard palate papules
It can occur at any age, but it is more common in young adults. The average age of onset is 30 to 40 years old. Children and newborns are rare. The age of onset is higher than that of those without diabetes. The gender difference is obvious. For 1:4, the disease is rare in the Eastern and black people. The lesions are mainly located on the side of the calf. The beginning is round, hard, dark red asymptomatic papules or plaques, one or several pieces, slowly developing. Mutual, ovoid or irregular, hard and plaque with clear and irregular edges, often brownish red or purple, flat or concave in the center, smooth and glassy, with obvious telangiectasia Fibrosis, appearance such as scleroderma, about 1/3 of the skin lesions may ulcer, the surrounding skin is normal, the lesions in the deep are often nodular, the surface of the skin is not changed, the damage can still affect the stock, About 15% of the calf flexion and the foot occur in the arms, trunk and scalp. In some cases, the lower extremities are not affected. The scalp can cause alopecia due to atrophy and scar-like skin lesions, often with hypopigmentation. Disease course Chronic, often slow development for several years, but also long-term prognosis is stationary or scarring.
Examine
Examination of progressive progressive necrosis
Histopathology: The main histopathological changes occur in the dermis, and progressive necrotic lesions and fibrotic areas with unclear boundaries can be seen, mixed with flaky lymphocytes and granulomatous infiltration. The tissue cells are fenced at the edge of the progressive necrotic area. Arranged, but unlike ring granuloma or rheumatoid nodules, epithelial-like cells can be clustered like tuberculosis or sarcoidosis, and foreign body giant cells and Langhansian giant cells can be seen. Thick, fibrotic, thinning or ulceration of the epidermis, skin appendages can also be destroyed, diabetic and non-diabetic in histology, the former is easy to see vascular lesions and fence-like changes around the necrotic area, the latter A common granulomatous reaction.
Diagnosis
Differential diagnosis of lipid progressive necrosis
The main diagnosis is based on the anterior scleroderma-like lesions of the lower extremity of the young and middle-aged women. It is not difficult to make a diagnosis in combination with histopathology.
Need to identify scleroderma, hard erythema, sputum mucinous edema.
