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

Lichen sclerosus and atrophicus (syn.: guttate scleroderma, white spot disease, white lichen of Zumbusch). The question of the independence of this disease has not yet been resolved.

Causes of lichen sclerosus and atrophicus

Most authors consider it as a separate nosological entity, others - as a variant of limited scleroderma and, finally, some consider it a disease that has an intermediate position between scleroderma and lichen planus, and when localized on the genitals, they identify it with kraurosis. According to MG Connelly and RK Winkelmann (1985), the similarity of the histological pictures of sclerosing lichen and lichen planus consists, first of all, in the presence of a strip-like infiltrate near the epidermis, the formation of blisters in the subepidermal region, the possibility of ulcerative changes. The description of various combinations of sclerosing lichen, lichen planus and focal scleroderma, including the presence of the listed forms in the same patient, gives reason to consider this disease from the standpoint of the "graft versus host" reaction.

Symptoms of lichen sclerosus and atrophicus

Clinically manifested by a rash of scattered or grouped, sometimes merging into small plaques of small papules, round or polycyclic outlines with a depression in the center, white with a livid shade of color, usually on the neck, shoulders, trunk, in the genital area. Comedo-like follicular keratotic plugs are found on the surface of individual elements. In rare cases, there are blisters, sometimes typical foci of superficial scleroderma are detected simultaneously. When localized on the genitals, the process can be complicated by the development of squamous cell carcinoma.

Pathomorphology. There is atrophy of the epidermis, hyperkeratosis with the presence of plugs in its depressions and the mouths of the 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 structureless, almost unstained. Below the edema zone there is a dense strip-like 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 deeper parts of the dermis. Electron microscopy revealed that the main changes are manifested by dystrophy of collagen fibers, in which transverse striae are not expressed, have the form of tubules. In fibroblasts, expansion of the endoplasmic reticulum cisterns and signs of decreased fibrillogenesis are observed. In some places, however, thin immature fibrils with a diameter of 40 to 80 nm are found. 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 familial cases of the disease, including monozygotic twins. An association of the disease with antigens of the HLA-A29, HLA-B44, HLA-B40 and HLA-Aw31 system has been noted. The possibility of the influence of hormonal disorders is indicated by the frequency of incidence mainly in women during menopause. Association with other autoimmune diseases (alopecia areata, hyper- and hypothyroidism, pernicious anemia, diabetes mellitus) indicates the presence of pathology of the immune system. In some patients and their first-degree relatives, circulating autoantibodies to the epithelium of the thyroid gland, gastric mucosa, smooth muscles, as well as antinuclear antibodies are detected. The absence of collagenase activity and the increase in the activity of collagen-inhibiting enzyme, as well as the suppression of elastase activity in the lesions, may be important in the development of the disease.

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