GEMODERMIYA (Greek haima blood + derma skin; synonym: hematodermatosis, gematodermatoza) — group of the dermatosis caused by malignant proliferation of cells of the hemopoietic bodies and reticular fabric.
The doctrine about G. arose at the end of 19 century. The first description of leukemia with damage of skin belongs to Besyadetsky (Biesiadecki, 1876). The generalizing works devoted to changes of skin at diseases of the hemopoietic system appeared a bit later [F. Pinkus, 1901, F. Paltauf, 1909]. Attempts of systematization of G. are made in Artst and Fus's works (L. Arzt, H. Fuhs, 1929), Shenghofa (S. Schoenhof, 1929), M. F. Sinani (1941). Further much attention was paid to damages of skin at reticuloses — to retikulodermiya which, according to Cesaria (A. Sezary, 1941, 1952), take the leading place in.
In the light of modern data on a hemogenesis a number of authors disputes expediency of the group term «gemodermiya» including very broad range of pathology; it is offered to use the term «retikulo hemoblastoses of skin» providing generalization kliniko-morfol. the signs characteristic of damage of skin both at various leukoses, and at reticuloses.
According to V. Ya. Arutyunov and P. I. Golemby (1964), G. make 1,65% of total number of a dermatosis; men are ill more often than women. The disease develops preferential at the age of 40 — 70 years.
The etiology and a pathogeny
Allocate nonspecific and specific G. Nonspecific G. are more often caused by the toksiko-allergic reactions developing against the background of process of disintegration of unripe blood cells (at a leukosis), and - are characterized by various erythematic, erythematic and squamous, papular, urtikarny rashes, is more rare vesicular or violent. At gistol, a research inflammatory process comes to light banal hron.
Nonspecific G.' emergence can be also connected with overwhelming dominance in blood of any type of cells; e.g., suppression of a poez of blood plates at hyperproduction of unripe leukocytes at a myeloleukemia causes a hemorrhagic enanthesis and mucous membranes.
Some authors expand nonspecific G.' framework, carrying in this group and infectious diseases of skin (pustulous, fungal, virus) arising at patients with leukoses against the background of decrease in immune responsiveness of an organism and also the changes of skin caused by effect of the medicines (toksikodermiya) used for treatment of malignant diseases of blood.
Specific G. are caused autokhtonno by the arising centers of an extramedullary hemopoiesis in skin, a source to-rogo are the low-differentiated cells reticular fabric (see), located under an endothelium of capillaries, around arterioles and venules, having potential differentiation potency and the proliferation similar to ability of cells of the hemopoietic bodies. Proliferation of the low-differentiated cells of reticular fabric often can be limited only to skin, or changes of skin are the leading symptom of a disease (primary reticulosis of skin, a reticulum cell sarcomatosis of skin, fungoid mycosis, angioretikulez Kaposha). Clinically specific G. are shown more often in the form of monomorphic rash (nodes or papules) or erythroses.
There is no general view on essence of these patol, processes. Many modern researchers consider G. as the peculiar tumoral process in reticular fabric differing in features inherent in it what confirm a wedge., morfol, and tsitol. data.
many classifications of G. Nekotorye from them, napr, offered Cesaria (A. Sezary, 1941, 1952), Dyuperra (V. of Duperrat, 1959), Kazal (R. of Cazal, 1952), Popov and Konstantinov (L. Popoff, A. Konstantinov, 1961), Popkhristov Were offered (P. Popchristov, 1968), are overloaded with details that reduces their practical value. A number of authors insufficiently reasonably expands G.'s framework, including in this concept fibroma, an angioma, tuberculosis, so-called exchange reticuloses, a pigmental small tortoiseshell, etc. In Goll's classification, Mallori (R. Gall, T. Mallory, 1942), Levera (W. Lever, 1958), Sira et al. (D. Cyr, 1966), Sutton (R. Sutton, 1956) do not enter primary reticulosis of skin, a reticulum cell sarcomatosis of skin, angioretikulez Kaposha and other forms
of G. V. Ya. Arutyunov and P. I. Golemba (1964) allocated proliferative G.' group including skin manifestations at leukoses (lymphadenoses, myeloleukemias, acute leukoses) and reticuloses which were subdivided into actually reticuloses (histiocytic, eosinophilic, lymphoreticuloses, etc.). tumoral (a reticulum cell sarcomatosis, etc.), granulomatoses (a lymphogranulomatosis, fungoid mycosis, an eosinophilic granuloma), exchange reticuloses (a disease to Gosha, Nimann's disease — Peak, Hend's disease — Schueller — Krischena, etc.), the secondary reticuloses mixed and transitional forms of reticuloses. Authors considerably expanded G.'s framework, having included in them also so-called not proliferative G. pathogenetic connected with such disturbances of a hemogenesis as anemias (hemolitic, Pernicious, drepanocytic), a Werlhof's disease, disproteinemias, etc.
A. A. Kalamkaryan (1969) allocated three groups G. on the basis of a complex a wedge., gistol, and tsitol. data. The first group included skin manifestations at leukoses; in the second — skin manifestations at a lymphogranulomatosis, Benye's sarcoidosis — Beck — Shaumanna, fungoid mycosis; the third group includes reticuloses of skin which number treat primary reticulosis of skin, a reticulum cell sarcomatosis of skin and angioretikulez.
A clinical picture
Changes of skin at an acute myeloleukemia (see. Leukoses ) more often nonspecific are also characterized by hemorrhagic rashes (petechias, ecchymomas), violent elements with hemorrhagic contents or multiple sites of a necrosis of skin and mucous membranes. Specific manifestations on skin are observed usually only at hron, a myeloleukemia and are characterized by the nodular or knotty rashes which are quite often ulcerating; histologically in infiltrate a large number of granular neutrophils in various stages of a differentiation is defined.
At hron, a lymphoid leukosis differ in a big variety of skin symptomatology. Nonspecific G. at hron, a lymphoid leukosis (see. Leukoses ) are characterized by pruriginous rashes, the Crimea it is quite often long the persistent skin itch, the small vesicular grouped rashes reminding herpetiform dermatitis of Dyuringa precedes (see. Dyuringa disease ). Specific changes of skin are shown in the form of an erythrosis, nodular or tumorous rashes. At localization of tumorous infiltrates on a face «facies leonina» reminding defeat at can be created to a leprosy (see). Histologically infiltrates at a lymphoid leukosis consist of lymphocytes and lymphoblasts.
At lymphogranulomatosis (see) the nonspecific changes of skin caused, as a rule, by the most severe pristupoobrazny itch therefore sites of a likhenization of skin form are more often observed. Also urtikarny, pruriginous, ekzemopodobny rashes are possible. Specific uzlovatoyazvenny rashes at a lymphogranulomatosis are observed seldom.
Damage of skin at sarcoidosis (see) polimorfno. Distinguish small - nodular, largely nodular, infiltrative, and also deep forms of defeat like sarcoids to Darya — Russia (see. to Darya-Russi sarcoid ).
At fungoid mycosis (see. Mycosis fungoid ) distinguish several wedge, forms among which the classical three-phasic form is most often observed. At this form of an enanthesis there pass three stages: erythematic, blyashechny and tumoral. Less often the erythrodermic form of fungoid mycosis and a form which is shown at once tumors meets. The last is identified by many authors with a malignant reticulosis. Histologically the granulematozny infiltrates consisting of histiocytes, unripe reticular macrophages with impurity of lymphocytes, neutrophils, eosinophils, plasmocytes, fibroblasts are characteristic of fungoid mycosis.
Reticuloses of skin (see) can be primary. At the same time changes in the hemopoietic bodies it is long do not come to light or are expressed poorly. Distinguish spotty blyashechnuyu, nodular, knotty versions. Secondary reticuloses of skin are specific manifestations of a reticulosis — the leukosis proceeding with the expressed primary defeats of the hemopoietic bodies. At reticuloses rashes are possible also nonspecific (hemorrhagic, urtikarny).
The reticulum cell sarcomatosis of skin belongs to tumoral reticuloses of skin. This form is regarded by Nek-ry authors as intermediate between true reticulosarcoma (see) and. At this type of a reticulosis damage of skin is characterized by multiple nodes, process is long does not go beyond an integument, further autokhtonno (but not metastatichesk that is characteristic for a reticulosarcoma) in patol, process internals are involved.
Angioretikulez Kaposhi (see. Kaposha sarcoma multiple hemorrhagic ) it is characterized by consecutive transformation of rashes like spots in small knots, plaques, nodes; infiltrate consists of the spindle-shaped cells relating to young fibroblasts, lymphocytes, histiocytes.
Some authors allow a possibility of transformation hron, an inflammatory dermatosis (e.g., psoriasis) in G., however the standard point of view in this question does not exist.
the Principles of treatment of gemodermiya consist in therapy of a basic disease with purpose of symptomatic outside means. Active treatment is good-quality the proceeding forms of a reticulosis of skin at early stages of development is inexpedient. During the progressing of a disease include cytostatic drugs, corticosteroid hormones in a complex of treatment. The choice of means and their combination are defined a wedge, a form and a stage of a disease: e.g., cytostatics are shown to patients with the developed picture of a disease and at emergence of signs of its progressing, corticosteroid drugs — at the widespread damages of skin which are followed by the ostrovospalitelny phenomena and also at various allergic (e.g., a toxidermia) complications. Cytostatic means (Dipinum, TIOTEF, fasfazin, Prospidinum, Photrinum, Phopurinum) in some cases are capable to suppress malignant proliferation of reticular fabric in skin. Their efficiency considerably amplifies in a complex with corticosteroid drugs. Cytostatics and endoksan in a combination with corticosteroids recommend sarcolysine, Dopanum, TEM only to patients at far come stages of fungoid mycosis and a reticulum cell sarcomatosis (existence of widespread tumoral cankers).
The local roentgenotherapy can be recommended in combination with cytostatic means or corticosteroid drugs a sick reticulum cell sarcomatosis and fungoid mycosis with tumoral defeats.
the Forecast depends on a form G. and a stage of process. — always potentially malignant disease. The forecast at primary reticulosis, fungoid mycosis of the first and second stage, an angioretikuleza is slightly better, than at a reticulum cell sarcomatosis, fungoid mycosis of the third stage and an acute leukosis with tumoral damages of skin.
selection of the pharmaceuticals providing long remissions of a disease and excluding possible stimulation of reticular fabric is important For prevention of a recurrence and G.'s aggravations.
Bibliography: Arutyunov V. Ya. and P. I. Gemodermiya's Golemba, M., 1964; Ashmarin Yu. Ya. Damage of skin and mucous membranes at reticuloses, M., 1972; Pototsky I. I. Reticuloses of skin, page 276, Kiev, 1972: Degos K. et Lortat-J acob E. Dermatologie, R., 1971; Gottron H. And. Retikulosen der Haut, in book: Dermatologie und Venerologie, hrsg. y. H. A. Gottron u. W. Schonfeld, Bd 4, S. 501, Stuttgart, 1960; W o z n i a k L. u. a. Histo-pathologie der Friihstadien der Retikulo-hamatodermien, Derm. Mschr., Bd 160, S. 529, 1974, Bibliogr.
A. A. Kalamkaryan.