RETICULOSIS OF SKIN (synonym: primary reticulosis of skin, reticuloendotheliosis of skin) — the disease caused by proliferation of tumor cells of an adenoid tissue, arising originally in skin and accepting in the subsequent system character.
The reticulosis of skin at different stages of studying was carried to gemodermiya, retikuloblastoza and other diseases that reflected the view of gistogenetichesky communication of R. which was outdate now to. with a reticular macrophage. Modern idea of R. to. as about a lymphoma of skin reflects its genetic linkage with a limfotsitopodobny cell. However the nature of proliferative cells is up to the end not established. Perhaps, R. to. — the heterogeneous group of diseases, one options a cut are caused by tumoral proliferation of V-lymphocytes, others — proliferation of cells of a monocytic row.
Etiology it is studied insufficiently.
Patogistologiya the first stage of R. to. it is characterized by the small perivascular proliferata located hl. obr. in upper parts of a mesh layer of a derma. Occasionally they meet in lower parts actually of a derma and even in a hypoderma, around hair follicles and sweat glands. Proliferata consist of lymphocytes and histiocytes. Proliferation of an endothelium and adventitia of blood vessels of a derma is characteristic.
In the second stage massive monomorphic kruglokletochny proliferata are found, sometimes about single huge kletkami-Mezhdu epidermis and the upper bound of a proliferat the strip of not changed collagen is visible, intensive growth of elements of walls of blood vessels and defeat of hair follicles of sweat and sebaceous glands is noted.
In the third stage are noted a sharp atipizm of cells, with a large number of mitoses, death of hair follicles, grease and sweat glands.
More often men of mature and advanced age are ill. River to. the increasing weight a wedge, changes is inherent. In its current it is possible to allocate three stages: the first, or nonspecific, the second, or a stage of the developed symptoms, and the third, or terminal. In the first stage of R. to. the general condition of patients is not broken. The disease is limited to damage of skin. At the same time the most frequent manifestations are flat, usually wrong, slightly infiltrirovanny plaques of brick-red color, as a rule with a weak scaly peeling are more rare than roundish or oval outlines (tsvetn. fig. 7). Hair on plaques are absent. Quantity of plaques variously — from single to multiple. They can arise on any site of skin, a thicket — in the field of a pelvic girdle.
R. is much more rare to. it is shown by papules and nodes. Papules (see) usually have a flatness, roundish outlines (tsvetn. fig. 8), rose-red coloring with a yellowish shade, the small size (5 — 15 mm), a hardly noticeable peeling. Rashes are similar to papular syphilides or displays of a papulonecrotic tuberculosis cutis. Less often papules have conical shape, horn spinules on a top (tsvetn. fig. 9), reminding small knots at Deverzhi's disease (see. Deverzhi disease ), are also located about mouths of hair follicles. Nodes (see. Node ) have the small size, a dense consistence and dark pink coloring with a brownish shade; they are as if inlaid in skin. Nodes can exist is long, sometimes up to two-three decades. Similar manifestations of R. to. describe as high-quality retikulomatoz Cesaria. Nodes, as well as papules, can be combined with plaques that is characteristic of R. to. In some cases damage of skin has character erythroses (see): a peculiar coloring of skin like hyperpegmentation at intensive suntan, scarcely noticeable infiltration, a scanty peeling and small islands of not affected skin (tsvetn. fig. 10).
The second stage is characterized developed a wedge, signs. Plaques become numerous and massive (tsvetn. fig. 11), in some cases with an exudative component. Sometimes in the field of axillary hollows, genitalias, the bottom of a stomach they are transformed to large tumorous educations — infiltrative and tumoral option (tsvetn. fig. 12). As a result of merge of numerous plaques or that is observed less often, owing to full generalization of initial changes the erythrosis in the second stage gets the completed wedge, a picture. At the same time distinguish dry, exudative and melanodermichesky types of an erythrosis. The general signs are a sharp thickening of skin due to infiltration, and sometimes and considerably the expressed puffiness (at exudative type), the exaggerated drawing of skin, a peeling, red skin color with various shades, usually cyanotic, palmar and bottom keratosis (see), hair loss, damage of nails. At melanodermichesky type of an erythrosis coloring of skin dark to gray and flaky (tsvetn. fig. 13), and such coloring can both accompany initial displays of a disease, and to arise at a long-term erythrosis. Papules and nodes in this stage increase in a size and number, become juicy, semi-spherical, can merge with each other. Nodes increase limf; there can be gepato-both a splenomegaly and other damages of internals; the general weakness, an indisposition, bystry fatigue, etc. join. From time to time fever develops, the expressed subjective complaints usually are absent.
In the third stage the numerous, quite often large juicy nodes of dark cherry color inclined to disintegration prevail. The formed ulcers differ in sharp morbidity. Nodes can arise both on probably healthy skin, and as a result of transformation of the previous rashes. Sharply the general state worsens, the cachexia, fever develops, changes of internals progress, and patients perish. Similar development of R. to. occurs slowly (from 10 to 30 years), and the general duration of a disease is defined by hl. obr. duration of the first stage. Against the background of the general progressing of symptoms separate rashes can be exposed to full regress, leaving sometimes a cicatricial atrophy.
River to. can clinically begin with any stage, including with the third. Nek-ry researchers such kind of R. to. consider as an independent nosological form — a so-called reticulum cell sarcomatosis of Gottron, or a reticulum cell sarcomatosis of skin. Also atypical forms P. are described to.: sklerodermopodobny, proceeding as acute varioliformny parapsoriasis (see), ulcer serpiginiruyushchaya, edges differs in rapid increase in usually single massive plaques due to peripheral growth with simultaneous exulceration in the center (tsvetn. fig. 14).
the Diagnosis is made on the basis a wedge, the picture confirmed gistol. research of the site of skin, and sometimes limf, nodes.
Differential diagnosis carry out depending on R.'s stage to. with various dermatosis: eczema (see), especially microbic, psoriasis (see), blyashechny parapsoriasis (see) and a premikotichesky stage of fungoid mycosis (see. Mycosis fungoid ), papular a syphilide of ohms (see. Syphilis ), a papulonecrotic tuberculosis cutis (see. Tuberculosis extra pulmonary ). The second and third stages of R. to. shall be differentiated from lymphogranulomatosis (see), sarcomas (see), lymphosarcomas (see) and terminal phase of fungoid mycosis.
Treatment, Forecast, Prevention
Treatment. In the first stage it is necessary to be limited to observance of the rational mode of work, rest and food, vitamin therapy and purpose of symptomatic outside means; in the second and third stages depending on a wedge, pictures use the complex of means including corticosteroids, cytostatics, derivatives of quinoline, and also a roentgenotherapy. In some cases perhaps operational excision of nodes.
Prevention is not developed.
See also Gemodermiya .
Bibliography: Ashmarin Yu. Ya. Damages of skin and mucous membranes at reticuloses, M., 1972; Kalamkoryan A. A. Clinic and therapy of reticuloses of skin, Yerevan, 1983; Carrhae I. and d river Limforetikulyarny diseases, the lane with English, M., 1980; Potekayev N. S., Ivanov O. L. and I with t r e about in and R. I. Erythrodermic form of primary reticulosis of skin, Vestn. dermas, and veins., jsft 8, page 6, 1978; Potekayev N. S., etc. To evolution of primary reticulosis of skin, in book: Aktualn. vopr. dermas. - veins., under the editorship of R. S. Babayants, page 129, M., 1971; Pototsky I. I. Reticuloses of skin, Kiev, 1972; In and of k e I. S. and. lake of Cutaneous malignant lymphoma, Cancer, v. 47, p. 300, 1981; Kresbach H. Anmerkungen zu den kutanen malignen Lymphomen, Z. Hautkr., Bd 55, S. 302, 1980; Lever W. F. a. Schaumburg-Lever G. Histopathology of the skin, Philadelphia — Toronto, 1975; W i 1 1 e m z e R. o. Primary lymphoblastic non-Hodgkin's lymphoma of the skin, Brit. J. Derm., v. 104, p. 333, 1981.
H. S. Potekayev.