Lichen sclerosing and atrophic: causes, symptoms, diagnosis, treatment

Lishar sclerosing and atrophic (syn: teardrop scleroderma, white spot disease, white lychen Tsumbusha). The question of the independence of this disease has not yet been resolved.

The causes of depriving sclerosing and atrophic

Most authors consider it to be a separate nosological unit, others as a variant of limited scleroderma and, finally, some consider it a disease having an intermediate position between scleroderma and red planus, and when identified on the genitals it is identified with kraurosis. According to MG Connelly and RK Winkelmann (19S5), the similarity of the histological patterns of sclerosing and red flat lichen consists primarily of the presence of a band-shaped infiltrate near the epidermis, the formation of blisters in the subepidermal region, the possibility of ulcerous changes. The description of various combinations of sclerosing lichen, red flat lichen and focal scleroderma, including the presence of the listed forms in the same patient, gives grounds for considering this disease from the standpoint of the "graft versus host" reaction.

Symptoms depriving sclerosing and atrophic

Clinically manifested by the rash of scattered or grouped, sometimes merged into small patches of small papules, rounded or polycyclic outlines with a westernization in the center, white with a livid shade of color, a bowl on the neck, shoulders, trunk, in the genital area. Comedon-like follicular keratotic plugs are found on the surface of individual elements. In rare cases, there are blisters, and sometimes typical foci of superficial scleroderma are identified. When localized on the genitals, the process can be complicated by the development of squamous cell carcinoma.

Pathomorphology. There is atrophy zidermisa, hyperkeratosis with the presence of congestion in its indentations and the mouths of hair follicles, in the basal layer - pronounced vacuolar dystrophy. Directly under the epidermis there is a wide zone of pronounced edema, in which the collagen fibers look unstructured, almost not colored. Below the zone of the edema is a dense band-shaped infiltrate consisting of lymphocytes and a small number of histiocytes. Collagen fibers in the lower part of the dermis are edematous, homogenized, intensively stained with eosin. Over time, subepidermal blisters are formed in the edema zone, the infiltrate becomes less intense, moves to the deeper parts of the dermis. Electron microscopy revealed that the main changes are manifested by the dystrophy of collagen fibers, in which transverse striations are not expressed, have the form of tubules. In fibroblasts there is an expansion of the endoplasmic reticulum and signs of decreased fibrillogenesis. In some places, however, thin immature fibrils with a diameter of 40 to 80 nm are detected. Destructive changes are also noted in elastic fibers.

Histogenesis is poorly understood. The role of genetic, hormonal, infectious and autoimmune factors is assumed. There are observations of family cases of the disease, including monozygotic twins. The association of the disease with antigens of the system HLA-A29, HLA-B44, HLA-B40 and HLA-Aw31 was noted. The possibility of the influence of hormonal disorders is indicated by the frequency of the incidence of predominantly female sex during the menopause. Association with other autoimmune diseases (alopecia areata, hyper- and hypothyroidism, pernicious anemia, diabetes mellitus) testifies to the presence of pathology of the immune system. Part of patients and their relatives of the first degree of kinship reveal circulating autoantibodies to the epithelium of the thyroid gland, the mucous membrane of the stomach, smooth muscles, and antinuclear antibodies. The absence of collagenase activity and increased activity of the collagen-inhibiting enzyme, as well as inhibition of elastase activity in the lesions, may be important in the development of the disease.

[1], [2], [3]