SCLEROMA

From Big Medical Encyclopedia

SCLEROMA (Greek. skleroma consolidation) — a chronic specific inflammatory respiratory disease.

As the separate nosological form is described in 1870 by H. Hebra and called by it a rhinoscleroma. H. M. Volkovich suggested to call this disease of a scleroma of respiratory tracts. The terms «rinofaringoskleroma», «laryngoscleroma» indicating only localization of process lost the value.

An etiology

the Activator C. it is considered to be a klebsiyella (see. Klebsiella ), H opened in 1882 by A. Frisch and grown up in pure growth in 1886. M. Volkovi-chem (Volkovich's stick — A. Frisch). However attempts to cause S. in an experiment by inoculation of this stick were not crowned with success.

The epidemiology

S. meets more often in the Western European part of the USSR (The Belarusian SSR, zap. part of the Ukrainian SSR), Central America and Indonesia; separate cases of S. are noted worldwide.

Ways and ways of infection are unknown. S.'s contageousness is not proved: the faces which are in long contact with the patient S. do not get sick. Also sporadic cases of S. do not find an explanation in the certain countries. However only sick S. of people since the activator C. is not found in the environment can be a source infektsin. There is an opinion that Volkovich's stick — Frisch can be formed in a human body owing to a mutation of others kleb-siyell.

A pathogeny

Volkovich's Stick — Frisch plays an essential role in a pathogeny patol. the process at S. which is result of an immune response of an organism on its presence. As manifestation of cellular immunity serves the macrophagic reaction leading to accumulation of round cells on the periphery of sticks of Volkovich — Frisch and, as a result of their phagocytosis and incomplete digestion, to formation of foamy cells of Mikulich, specific to S. In sick S.' blood specific antibodies come to light. Artificial immunity is noted.

Pathological anatomy

Patol. process at S. extends on the course of respiratory tracts, including a threshold of a nasal cavity, the nasal courses, area of a postnaris, a nasopharynx (a nasal part of a throat, T.), stomatopharynx (oral part of a throat, T.), throat, trachea, bronchial tubes. Also parts of an outside nose, a lip, corners of a mouth, a gum, language, a soft and hard palate can be involved. Refer S.'s defeat to rare localizations of adnexal bosoms of a nose (okolono-sovy bosoms, T.), nasal ducts, conjunctiva of eyes, eustachian (acoustical T.) pipes, middle ear, outside acoustical pass. Destruction is described by skleromny infiltrate of separate face bones. The most expressed changes note in places where there are obstacles to the movement of an air stream — transition of a threshold to the nasal courses (a threshold of a nasal cavity), a postnaris, a cavum infraglotticum of a throat, bifurcation of a trachea.

Gross skleromny lessions represent diffusion or nodal thickenings of skin and mucous membrane. In the beginning they have a soft consistence, and in the subsequent get cartilaginous density, are not inclined to an ulceration. Skin over skleromny infiltrates smooth, brilliant, motionless, is deprived of glands and hair. A mucous membrane in sites of defeat bright pink or dark red, melkobugristy, firm. Occasionally on it superficial ulcerations, crusts meet. On a section the skleromny centers have an appearance of yellowish consolidations, to-rye I. Mikulich compared to old fat. The cicatricial fabric developing on site infiltrates roughly deforms a mucous membrane that can lead to a stenosis of respiratory tracts.

Fig. 7 — 8. Microdrugs of a skleromny granuloma of a stomatopharynx. Fig. 7. Under the thinned atrofichny epithelium (a dark strip in an upper part of drug) growth of granulyatsionny fabric with a large number of cells of Mikulich is visible (separate groups of cells are specified by shooters); coloring by gematoksilii-eosine; X 160. Fig. 8. Erythrocytes (orange color) and numerous cells of Mikulich are under review visible (are painted in dark color; kernels — black, cytoplasm lighter). Black pointed educations in cytoplasm of some cells of Mikulich — Volkovich's sticks — Frisch (two such cells are specified by shooters); coloring by azur-eosine: x 900.

At gistol. a research in skle-romny infiltrates and nodes specific granulyatsionny fabric is found, to-ruyu quite often call skleromny granuloma (see). It represents young connecting fabric (see) with vessels of capillary type, around to-rykh lymphoid cells, histiocytes, a large number of plasmocytes and peculiar large cells with light foamy cytoplasm — Mikulich's cells are visible, to-rye diffuzno infiltrirut granulyatsionny fabric or are located with groups (tsvetn. fig. 7). In Mikulich's cells, and sometimes and out of them at use of special colourings Volkovich's stick — Frisch comes to light (tsvetn. fig. 8). Eosinophilic and neutrophilic leukocytes (granulocytes) are present at rather small amount. Existence in specific granulyatsionny fabric of the so-called russelevsky little bodies known also as Pellizzari and Kornil's hyaline little bodies is characteristic.

Most of researchers connect an origin of cells of Mikulich with presence at monocytic macrophages of sticks of Volkovich — Frisch. With the help submicroscopy (see) in cytoplasm of phagosomas of cells of Mikulich Volkovich's sticks — Frisch both intact, and being at different stages of digestion are found. Availability of foamy cytoplasm of a cell of Mikulich is explained with accumulation in phagosomas of undigested bacterial glikozaminoglikan, to-rye, increasing osmotic pressure, promote vacuolation and a rupture of phagosomas.

An epithelium over skleromny granulations in various degree infiltrirovan neutrophilic leukocytes. In cells of an epithelium signs are noted vacuolar dystrophy (see), the papillary layer of skin is maleficiated, is exposed to an atrophy, edges is observed also in grease and sweat glands, follicles of hair. The cylindrical ciliary epithelium of mucous membranes is replaced multilayer flat, sometimes with patol. keratinization like a hyperkeratosis (see. Keratoza ) and a parakeratosis (see), forms akantotiches-ky immersions in specific granulyatsionny fabric, sometimes with atipichesky reactive growths. Over large hilly nodes thinning of an epithelial cover to 2 — 3 rows of the flattened cells can be observed.

The essential place in development of skleromny defeats is taken by the phenomena sclerosis (see), caused by growth and maturing of specific granulyatsionny fabric. Formation of collagenic fibers in the form of bunches begins in deep departments of skleromny granulations and goes towards a surface. At the same time granulyatsionny fabric is divided into the separate islands captured by the rough, quite often hyalinized bunches collagen (see). Along with it the new growth of elastic fibers is noted. Disappearance of specific granulyatsionny fabric at treatment by streptomycin is also connected with strengthening of a sklerozirovaniye, at the same time first of all reduction of quantity, and then disappearance in granulyatsionny fabric of cells of Mikulich is observed.

At a long current of S. specific morfol. changes are sometimes observed in regional limf, nodes. In internals of any specific manifestations of a scleroma it is not revealed.

The clinical picture

S. proceeds for years and decades. The incubation interval lasts several years. Most of patients get sick at the age of 15 — 20 years, cases and at children's age meet.

During S. allocate three periods: preclinical, in Krom positive takes serol come to light. researches, but still there are no wedge, manifestations; the clinical, characterized explicit wedge, manifestations and positive serol. reactions; residual, in Krom only the residual phenomena (hems, an atrophy of mucous membranes) are observed.

Clinically S. proceeds in two forms — productive and dystrophic, to-rye in some cases are combined. Emergence of the granulematozny changes increasing or ekzofitno in the form of magnificent, sometimes tumorous educations, or endofitno — under a mucous membrane, in the form of dense infiltrates is characteristic of a productive form. Granulematozny growths have no tendency to disintegration and over time are replaced with dense hem (see).

Fig. 1. A face of the patient with a scleroma with damage of a nose.
Fig. 2. A face of the patient with a scleroma with infiltrates in the field of the lacrimal openings.

Skleromny productive changes in area of a nose find in the field of a threshold of a nasal cavity more often, to-ry is annularly narrowed, and also extend to an upper lip and wings of a nose (fig. 1). On a nasal duct process extends to area of the lacrimal openings (fig. 2); in isolated cases the eye-socket is surprised. The infiltrates located in the field of a postnaris kontsentricheskn narrow their gleam, spreading to a nasopharynx, is funneled narrow se or form kulisovidny tyazh. The soft palate involved in process is tightened up, the uvula is unbent and soldered to an upper surface of a soft palate. Sometimes skleromny infiltrates pass on palatoglossal and palatopharyngeal handles, almonds, language, concentrically narrowing a pharynx, occasionally are found in anticipation of a mouth. In a throat granulematozny changes meet in any its site; the most typical is formation of valikoobrazny infiltrates in a cavum infraglotticum. Infiltrates most often are in a trachea and bronchial tubes on Kiel tracheas, but happen also on other sites.

Productive changes in respiratory tracts lead to narrowing of their gleam, than the most typical complaints of patients to difficulty of nasal breath and breath in general, change or loss of a voice, difficulty of swallowing are caused.

The dystrophic form C. proceeds with a mucosal atrophy of respiratory tracts, its dryness, decrease in sensitivity, education viscous separated and crusts with off-flavor, however not so sharp, as at to an ozena (see). Patients complain of feeling of dryness in a nose and a throat.

At sick S. the hypoadrenalism, a hypovitaminosis are noted. Patients complain of the general weakness, fatigue, drowsiness, disturbance of appetite, a hypomyotonia connected with the oxygen insufficiency caused both by disturbance of external respiration, and an anoxemia against the background of the functional insufficiency of marrow leading to hypochromia anemia.

Complications Pages are nonspecific dacryocystites (see. Dacryocystitis ), decrease in sensitivity of a cornea, decrease in dark adaptation (see. Gemeralopiya ), narrowing of peripheral sight, a tracheobronchitis (see. Tracheitis ), bronchiectasias (see), emphysema of lungs (see), a pneumosclerosis (see).

The diagnosis

the Diagnosis is established on the basis of the specific changes found at the research of respiratory tracts including a trakheobronkhoskopiya it is desirable optical (see. Bronkhoskopiya ). Great value, especially at early stages of a disease, serological researches represent: reaction of binding complement (see) with skleromny antigen (a heated-up microbic emulsion of a stick of Volkovich — Frisch) and reaction agglutinations (see) acapsular option of this stick. The diagnosis confirms detection of cells of Mikulich in tsitol. and gistol. drugs, and also identification of a stick of Volkovpcha — Frisch in separated respiratory tracts.

Radiological, according to S. I. Vulfson and Ya. G. Dillon (1926), the centers of calcification in a zadnenizhny third of a thyroid cartilage, narrowing of an air column in a cavum infraglotticum are characteristic of S. of a throat; ossification of cartilages in the form of the centers with the shoots confused in a ball, loops of the stiffened masses in the field of plates of a thyroid cartilage.

At tomographies (see) or a contrast trakheobronkhografiya (see. Bronchography ) it is possible to reveal concentric narrowing of a throat and trachea up to their sharp stenosis (see. Laryngostenosis , Tracheostenosis ).

In a crust, time in connection with development of more informative endoscopic methods of a research rentgenol. S.'s diagnosis does not find for a throat broad application.

Differential diagnosis carry out with an ozena (see), tuberculosis (see), syphilis (see), a leprosy (see), Wegener's granulomatosis (see Wegener a granulomatosis), and also malignant new growths.

Treatment

Treatment includes first of all use of antibiotics depending on sensitivity of Volkovich's sticks to them — Frisch, sowed at this patient. The first course of an antibioticotherapia shall be the most long. Penetration of an antibiotic into depth of a granuloma is promoted by use of 30% of solution of a dimethyl sulfoxide in the form of applications on area of granulematozny growths, pyrogenal. Granulematozny growths delete in the operational way, sites of narrowing buzhirut. Atrophic sites of a mucous membrane grease with yodistomaslyanisty drugs, apply inhalations by alkaline solutions. At deterioration in the general state vitamin therapy, inhalations of oxygen, purpose of co-carboxylase are shown.

Radiation therapy (see) apply in combination with operational treatment, sometimes use and as an independent method. It is effective at infiltrative forms, especially in early stages of a disease; radiation usually leads to a full or partial rassasyvaniye of specific skleromny infiltrates or to their bystry scarring. The best results are observed at superficial and limited defeats, the worst — at diffusion and infiltrative forms of a disease, and at the expressed atrophic or cicatricial changes the positive effect is absent. At the severe defeats which are followed by exhaustion of patients, radiation therapy is contraindicated.

The forecast

the Forecast for life at early diagnosis and timely treatment favorable. Working ability of patients depends on extent of damage of airways and on localization in them specific changes.



Bibliography: Barilyak I. R. Changes of an organ of sight at patients with a scleroma „Zhurn. ushn., nose. and throats, Bol., No. 4, page 69 „1974, bibliogr.; Bar and l R. A. yak and With and x e l and sh in and l and N. A. Skleroma, Kiev, 1974, bibliogr.; M. K. Patologogistologicheskiye's distance of change of blood vessels in the fabrics struck with a scleroma, Zhurn. ushn., nose. and throats, Bol., No. 2, page 23, 1968; To r and with and l N and-kov A. P. and And z r and and t e l N. A. Skleroma, Minsk, 1971, bibliogr.; Their m and y l about in with to and y S. V. Skleroma of respiratory tracts, M., 1959, bibliogr.; The multivolume guide to pathological anatomy, under the editorship of A. I. Strukov, t. 9, page 515, M., 1964; Bridge S. I. Radiodiagnosis of a scleroma of a trachea and bronchial tubes, Kiev, 1965, bibliogr.; Problems of a skleromny infection, under the editorship of B. Ya. El-bert, Minsk, 1957; The Scleroma, under the editorship of. A. I. Kolomiychenko, Kiev, 1959; H e Ь-r H. tfber ein eigenthiimliches Neugebilde an der Nase: Rhinosclerom, Wien. med. Wschr., s. 1,1870; HoffmannE. O., L about o s e L. D. a. H and of k i n J. C. The Mikulicz cell in rhinoscleroma, Amer. J. Path., v. 73, p. 47, 1 973; T o p p o-z a d a H. o. The tunica progria in rhinoscleroma, Acta otolaryng. (Stockh.). v. 91, p. 595, 1981.


R. A. Barilyak; V. P. Bykova (stalemate. An.), A. N. Kishkovsky (I am glad.).

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