LYMPHOGRANULOMATOSIS PAKHOVY

From Big Medical Encyclopedia

LYMPHOGRANULOMATOSIS PAKHOVY (lymphogranulomatosis inguinalis; lat. lymphogranuloma, lymphogranulomat[is] + - osis; synonym: the venereal lymphopathy, the fourth venereal disease, Nicol's disease — Favra, a lymphogranuloma inguinal, a subacute pakhovy paradenitis, pakhovy purulent mikroporadenit) — the venereal disease meeting mainly in the countries with tropical and subtropical climate. A number of researchers suggested to call a disease «a venereal lymphopathy» to exclude the term «granuloma» or «lymphogranuloma», because of to-rogo given nozol, the form can be confused with other diseases, in particular with a venereal granuloma (see. Granuloma venereal ).

The disease was for the first time described in 1912. G. A. Rost and as independent nozol, the form is allocated in 1913 by Durant, Nikola and Favr (J. Durand, J. Nicolas, M. to Favre). In 1923 Mr. G. Gamna opened cytoplasmic inclusions in the infected cell, morfol, the structure to-rykh was described after Findlay (G. M of Findlay, 1933) and Y. Miyagawa with sotr. (1935). In 1930 Hellerstrem and Vassen (S. G. A. Hellerstrom, E. Wassen) established filterability of the activator.

Infection occurs usually sexually and much more rare household; isolated cases of professional infection, in particular surgeons are described.

Etiology

Activator L. the item is defined as a galproviya or a chlamydia; synonym: miyagavanella, bedsoniya. He is a representative closely related to bacteria of the group of microorganisms which is earlier considered as group of large viruses of a psittacosis — a venereal lymphogranuloma — trachoma (PLT) carried in system of a microcosm to an independent order of Halprowiales (A. A. Shatkin, 1965) or Chlamydiales [L. A. Page, 1966]. This order combines gram-negative obligate parasites of eukariotsit of the prokariotny nature.

Fig. 1. The infected cell (at the left) with the cytoplasmic inclusion (is specified by an arrow) containing the activator of an inguinal lymphogranulomatosis (in a light microscope). Coloring across Romanovsky — to Gimza; x 2000.
Fig. 2. The diffraction pattern of morphological structures of the activator of an inguinal lymphogranulomatosis in cytoplasmic inclusion: 1 — initial, 2 — intermediate, 3 — elementary little bodies; x 90 000.

Activator L. the item has structural and chemical properties and a difficult cycle of intracellular development, similar to gram-negative bacteria. An infectious form is the roundish elementary little body to dia. 0,25 — 0,3 microns limited to a cell wall and a cytoplasmic membrane and containing electronic and dense nucleoid and ribosomes. The elementary little body gets into cytoplasm of a cell in a phagocytal vacuole, in a cut and there are consecutive stages of reproduction of a microorganism to formation of noninfectious vegetative forms — so-called initial (reticular) and intermediate little bodies and transformation of the last to new generation of infectious elementary little bodies. The activators L breeding in a vacuole. items form cytoplasmic inclusions (fig. 1 and 2). The development cycle proceeds during the 48th hour. Elementary little bodies are painted by Romanovsky's method — Gimza and Makkiavello in a magenta color, vegetative forms — in blue. All forms contain DNA and RNA.

Activator L. the item is cultivated in cultures of cells of vertebrata, in epithelial cells of covers of vitellicles of the developing chicken embryos, in macrophages, in an organism of white mice at intracerebral and intranasal infection. Has pathogenicity for Guinea pigs, monkeys, cats, dogs. Also the asymptomatic carriage is characteristic. It is highly sensitive to drying, influence of UV rays, termolabilen. It is inactivated at t ° 50 ° in 30 min. 0,1% by solution of formalin or 0,5% solution of phenol — in 24 hours, ether and ethanol — in 30 min. at t ° 18 °. It is long remains at t ° — 70 ° and after lyophilizing. Reproduction is suppressed with antibiotics (tetracyclines, Makrolida are most effective) and streptocides. The microorganism contains the lipoglikoproteinovy antigen, group-specific for galproviya (chlamydias), revealed in RSK and others serol, reactions, species-specific antigen of the proteinaceous nature and a toxic factor. Among the strains of the activator L circulating in the nature. by the item it is defined 3 serol, option. Differentiation and identification is carried out on biol, to characteristics.

Patogistologiya

Gistol, a picture depends on the period and clinic of a disease. Primary defeat — inside - or a subepidermal bubble. Cellular infiltrate accumulates in surface layers of skin, consists of macrophages, lymphocytes with impurity of polymorphonuclear leukocytes. In process of education erosion (see), and then breaking up ulcers (see) in the center of infiltrate there is a necrosis, accumulation of lymphocytes and polymorphonuclear leukocytes with epithelial cells on the periphery. There are colossal cells inclined to focal accumulation on type inf later. granulomas (see).

Changes limf, nodes are characteristic. Macroscopically they color, red with a grayish shade, are soldered among themselves, on a section — small abscesses, ochazhka of a necrosis to a typical radial outline. The microscopic picture corresponds subacute or hron, to lymphadenitis with considerable destruction of structure limf, nodes. In fabrics around struck limf, nodes — hyalinizations of vessels, multiple blood clots, a hyperplasia of an endothelium, perivascular infiltrate. During the formation of a genitoanorektalny syndrome development of granulyatsionny fabric with infiltration by macrophages, plasmocytes, polymorphonuclear leukocytes is characteristic.

Clinical picture

Fig. 3. An ulcer on a prepuce of a penis at an inguinal lymphogranulomatosis

The incubation interval is more often than 1,5 — 2 weeks. In typical cases distinguish three consecutive periods. Primary (limfogranulematozny chancre): on site implementations of the activator, i.e. in the field of generative organs (perhaps extragenital defeat — lips, an oral cavity, language, etc.), there is a single bubble (more rare a papule) which is quickly transformed to an erosion, and then to an ulcer to dia, to 1 — 3 cm of the wrong roundish or oval outlines, superficial, with an uneven bottom (fig. 3), covered with pus grayish and dim, sometimes a yellowish-greenish shade. Existence of an ostrovospalitelny rim and absence characteristic of a hard ulcer is typical (see. Syphilis ) consolidations in the basis. Usually in 7 — 9 days against the background of the outlined tendency of an ulcer to spontaneous healing gradual increase, consolidation and morbidity regional limf, nodes (at men is more often inguinal, at women — a small pelvis) is observed.

1,5 — 2 months later after infection the secondary period is caused by generalization of process: limf, nodes increase even more, are condensed and as a result of distribution of inflammatory infiltrate (periadenitis) merge among themselves in a conglomerate, painful at a palpation, massive with a chicken skin. Further in the thickness of a conglomerate the numerous centers of a softening, fistulas and fistulas develop, from to-rykh pus, sometimes with impurity of blood is emitted. At the torpid course of process sclerous changes of the struck fabrics, formation of commissures and hems are expressed that brings to to a lymphostasis (see) and to hypostases in the field of generative organs.

In the secondary period of L. items, except defeat regional limf, nodes, in patol, process can be involved deep limf, nodes of a small pelvis, innidiation of an infection from the area of genitalias to sites (metastatic buboes of upper extremities), damage of internals, joints, veins (phlebitis of bodrenny veins), and also an epididymite, an adnexitis, an iritis, conjunctivitis, a keratitis and even meningitis, remote from primary center of defeat, is possible. Cases of emergence on skin of sick L are described. the item of the rashes of allergic character reminding a small tortoiseshell, a knotty or mnogoformny exudative erythema, rash at scarlet fever.

The tertiary period of a disease is usually shown in 1,5 — 2 (sometimes a bit later) and the hl is expressed. obr. in development of a so-called genitoanorektalny syndrome — the heavy fistulose and commissural process developing as a result of distribution of inflammatory process on a crotch and perianal area with development proctitis (see) and paraproctitis (see). The most precursory symptom of a genitoanorektalny syndrome — nagging pains in a crotch, scanty sanious purulent discharges from an anus. At a rektoromanoskopiya — hypostasis and a hyperemia, especially lower part of a rectum, an erosion with sukrovichnognoyny separated, bleeding and morbidity at the slightest stretching of a mucous membrane. Cicatricial and sclerous changes in a rectum lead to development of the strictures covering usually koltsevidno a rectum that can lead to almost full closing of a gleam of a gut.

The proctitis and a paraproctitis can be followed by abscessing with the subsequent ulceration, formation of fistulas in the field of an anus, a crotch, generative organs, etc. Women have an exulceration and the subsequent scarring of the centers of defeat quite often lead to strictures of a vagina, especially in the field of an entrance, to considerable deformations of small and big vulvar lips, a clitoris.

In tertiary, is more rare in secondary, the period of a disease the general phenomena are possible: weakness, loss of appetite, headaches, weight loss, and also joint pains (late limfogranulematozny polyarthritis), acceleration ROE, leukocytosis, anemia.

Diagnosis the wedge, this, characteristic of every period of a disease is put on the basis. Besides, diagnosis of L. the item can be confirmed a lab. data: detection of the activator in smears prints of contents of buboes, its allocation at crops in the developing chicken embryos and cultures of cells, identification of group-specific complement-linked and type-specific fluorescent antibodies, hypersensitivity of the slowed-down type by means of an intracutaneous test (Frey's reaction). Allergen for Frey's reaction is prepared from pus of buboes or from vitelline cultures of the activator L. the items cleared by differential centrifuging. On 0,1 ml specific and control (the free of activator L. items) antigens vnutrikozhno enter into the flexion surfaces of forearms. The result is considered in the 48th hour. Reaction is estimated as positive at emergence of a papule or consolidation to dia. 5 mm and more and in the absence of consolidation in an injection site of control antigen. The size of a surrounding erythema is not considered. RSK and Frey's reaction at L. the item confirms a wedge, the diagnosis to 75% of cases. Formolovy reaction Ghat — Papakostasa who was earlier applied to confirmation a wedge, the diagnosis has no specificity. Complex lab. diagnosis allows to reveal and latent forms of an infection.

Differential diagnosis carry out with syphilis (see), soft chancre (see), a lymphoreticulosis, a so-called disease of cat's scratches (see. Benign inoculation reticulosis ).

Treatment

is Applied by streptocides (Norsulfazolum, Sulfadimezinum, etc.) on 1,5 g 3 times a day during 2 weeks, and then on 1,0 g 3 times a day during 3 weeks. A course of treatment — 5 weeks. In the presence of profound fibrous changes, and in particular strictures, in 2 weeks purpose of a repeated course of treatment is shown. Antibiotics of a tetracycline row (tetracycline, Oxytetracyclinum), and also erythromycin, Oleandomycinum and levomycetinum are effective. At accession of a consecutive staphylococcal infection the antibiotics of penicillinic group steady against a penitsillaza (Oxacillinum, Methicillinum) are shown. If there are fistulas spoiling hems, strictures use of an aloe, vitreous, lidaza, bougieurage of an urethra, in the absence of effect — an operative measure is shown.

Forecast concerning life usually favorable. The disease leaves durable immunity and reliable cases of repeated infection is not described. In the started cases secondary anemia, exhaustion, sepsis with a lethal outcome are possible.

Prevention: early detection and sanitation of sources of infection, control of completeness and quality of treatment; to the persons which had sexual contact with sick L. the item with the preventive purpose can be carried out an antibioticotherapia (tetracycline during 2 weeks).



Bibliography: Babayants R. S. Skin and venereal diseases of tropical countries, M., 1972; Laboratory diagnosis of viral and rickettsial diseases, under the editorship of E. Lennet and N. Schmidt, the lane with English, page 708, M., 1974; M and y with yu to A. P. Pathological anatomy, pathogeny and classification of a venereal lymphogranuloma, Vestn, dermas, and veins., No. 4, page 83, 1962; Durand M., Nicolas J. et F a v r e M. Lymphogranulomatose inguinale subaigue d'origine genitale probable, peut-etre ven^rienne, Bull. Soc. med. H6p. Paris, t. 35, p. 274, 1913; Manson’s tropical diseases, ed. by Ch. Wilcocks a. P. E. C. Manson-Bahr, Baltimore, 1972; W i 1 1-cox R. R. Lymphogranuloma venereum, in book: Morton R. S. a. Harris J. R. W. Recent advances in sexually transmitted diseases, p. 188, Edinburgh a. o., 1975, bibliogr.


P. S. Babayants; A. A. Shatkin (etiol., mt. issl.).

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