Granulomatous loose skin
Introduction
Introduction to granulomatous relaxation skin Granulomatous slacks are a rare subtype of cutaneous T-cell lymphoma with low malignancy. In 1973, Convit and Kerdel were first reported as progressive atrophying chronic granulomatous dermohypodermatitis (progressive atrophying chronic granulomatousdermohypodermatitis). Skin damage occurs in the armpits and groin, and is rare in the chest, ribs, forearms, thighs, feet, jaw and back. basic knowledge The proportion of sickness: 0.0001% - 0.0002% Susceptible people: no special people Mode of infection: non-infectious Complications: emphysema
Cause
Granulomatous relaxation skin cause
(1) Causes of the disease
The cause is not clear.
(two) pathogenesis
The pathogenesis is still unclear.
Prevention
Granulomatous relaxation skin prevention
Regular exercise to enhance physical fitness.
Complication
Granulomatous relaxation skin complications Complications emphysema
Emphysema is a common complication of granulomatous loose skin, the degree of lesions are not equal, light can not cause symptoms, and severe cases are the main cause of death. The severity of emphysema is consistent with the extent of skin lesions. Sometimes emphysema is the first symptom. Patients with obvious emphysema may have difficulty breathing, and are more obvious when lying down. Pulmonary function tests were clearly abnormal.
Symptom
Granulomatous relaxation skin symptoms common symptoms ring granuloma neck wrinkles papules nodular nodule abscess skin heterochromia
Insidious onset, skin damage occurs in the armpits and groin, rarely seen in the chest, ribs, forearms, femurs, feet, chin and back, 3 cases reported in China, 1 case occurred in the palm, can affect function, 1 For example, after the skin test of leprosy, 1 case occurred after a foot injury. The skin damage started from reddish to purple rash, pimples and plaques, the surface was bright, often lost skin, with a small amount of scales. Significant telangiectasia, clear boundary, solid texture, a few can be ulcerated, after the central atrophy, soft, sagging, wrinkling, subcutaneous blood vessels become obvious when atrophy, 7 cases (35%) reported to be Associated with Hodgkin's disease (HD) skin and/or lymph node damage, the other 3 (15%) developed systemic non-Hodgkin's lymphoma.
Skin lesions are large invasive red plaques with cutaneous heterochromia, which can last for many years. Due to the release of elastic fibers in the whole dermis during the pathogenesis, the skin atrophy and relaxation is drape and crease, which can be seen in the axilla and groin.
Examine
Examination of granulomatous loose skin
Histopathology: the earliest dermal superficial or full-thickness perivascular lymphocytes infiltrated, visible scattered in giant cells, epidermal keratosis, slightly hyperplasia, mild sponge formation, single lymphocyte infiltration in the lower part of the epidermis, when fully developed, It is characteristic, dense and dense, and small lymphocytes slightly distorted in the nucleus diffuse into the whole dermis and subcutaneous tissue, and can penetrate into the epidermis. It is often distributed in the papillary layer, edema or fibrosis of the dermal papilla, in the epidermis. Although single or small clusters of lymphocytes infiltrate, the formation of sponge is disproportionate or extremely slight. Another feature is the infiltration of a large number of multinucleated giant cells. The cytoplasm of giant cells is abundant, the cell boundaries are clear, and there are many nuclei (10 to 90). They are round or oval, closely arranged with each other, and phagocytic lymphocytes are seen in giant cells. Some giant cells are surrounded by macrophages arranged in a ring, and granulomas are often formed by macrophages and scattered giant cells. , granuloma or fibrosis between granuloma, 1 case of granulomatous arteritis, lymphocytes and giant cells in the arterial wall, but in most cases not involving moderate Large large blood vessels, the elastic fibers in the whole dermis almost disappear completely, and elastic fiber fragments are sometimes seen in the early giant cells.
The whole dermis and even the subcutaneous tissue are seen to infiltrate the lymphatic tissue cells, and the large multinucleated giant cells form a focal granuloma to form a granuloma. The histological features of the disease can completely replace the fat lobule, the elastic fiber cells are significantly increased, and the elastic fibers in the inflammatory zone are lacking. Lymphocytes are seen in the cytoplasm of multinucleated giant cells.
Under electron microscopy, multinucleated giant cells have obvious ciliated processes, which contain many lymphocytes. The lymphocytes seem to be well preserved, surrounded by the cell membrane of tissue cells, suggesting pseudophagocytosis, and multinucleated giant cells are in close contact with tumor lymphocytes.
Immunohistochemistry: small lymphocytes showed T helper cell phenotype, ie CD3, CD4, CD8-, CD30-, multinucleated giant cells positive for lysosome, S-100 protein, CD68, negative for MAC387 and vimentin In most cases, TCR gene cloning rearrangements can be shown.
Diagnosis
Diagnosis of granulomatous relaxation skin
diagnosis
In the dermis of the disease, multinucleated giant cell infiltration of diffuse phagocytic lymphocytes, mild atypical lymphocytes, epidermal and Pautrier microabscess accumulation and extensive destruction of elastic fibers are diagnostic and sometimes need to be identified with the following diseases:
1 granulomatous MF: no special predilection site, skin lesions are not slack, lesions are focal, giant cell nuclei generally 5 to 10, not as good as GSS, giant cell nuclei can reach more than 40, elastic fibers only partially disappear.
2 granulomatous skin diseases: such as sarcoidosis, reticular cell tumor and (or) multicentric reticulosis, as well as ring granuloma and even infectious skin diseases, combined with clinical manifestations are not difficult to distinguish.
3 Acquired skin relaxation: The boundary of skin damage is less clear. In the early stage, a small amount of neutrophils are seen. The elastic fibers are fragment or granular, and 4 spots are atrophied. The lesions can be outwardly bagged after finger pressure.
Differential diagnosis
1. Skin relaxation (cutis laxa) also often shows loose skin wrinkles, but no infiltration.
2. Granulomatous MF granulomatous relaxation The histological characteristics of the skin are very special, manifested as a single distribution of multinucleated giant cells in the dermal lymphoid cell infiltration, usually not formed in the granulomatous MF Epithelial-like or granulomatous nodules were seen, especially in the presence of obvious elastogenic fibroblasts. In addition, although mild single lymphocytes were found to be epidermal, they lacked intraepidermal microabscess.
3. Sarcoidosis is a nodule formed by epithelioid cells and a small number of lymphocytes, usually without elastic fiber rupture.
