Scleroma (skleromny disease) — the chronic disease of an infectious etiology which is characterized by inflammatory changes in a wall of airways with formation of granulomas. The clinical picture of a scleroma depends on its localization and can be shown by various violations of breath, disorder of voice function, a nose congestion, unpleasant feelings in a throat. Diagnosis of a disease is based on detection of a stick of Frisch-Wolkowitsch in dabs and separated, identification of specific cells of Mikulich in the bioptat taken during endoscopy from the place of skleromny defeat. Medical actions at a scleroma include anti-inflammatory and etiotropny therapy, local X-ray therapy, a buzhirovaniye of stenoses of airways, expeditious removal of granulomas and cicatricial fabrics.
The scleroma is widespread worldwide in a type of the endemic centers. The scleroma meets in Central and Eastern Europe, Central America, Indonesia, India more often. The area, endemic on a scleroma, usually represents the lowland with swamps and a light forest. Among the diseased with a scleroma workers of agriculture with low economic and social situation prevail. At women the scleroma is observed more often than at men. Often the scleroma strikes several members of one family. Most often it is diagnosed at the age of 15-20 years.
The scleroma has a slow current and can last for decades. Depending on localization of skleromny granulomas experts in the field of otolaryngology, stomatology, ophthalmology, pulmonology are engaged in diagnostics and treatment of a disease.
Etiology and pathogenesis of a scleroma
The scleroma represents an infectious disease. Its activator is Frisch-Wolkowitsch's stick, and an infection source — the sick person. The otolaryngology still has no exact data on the mechanism of transfer of a scleroma. Most of researchers assume a contact way of infection with a scleroma that is confirmed by its emergence at members of one family. However cases when the people who are in contact with the patient did not get sick with a scleroma are known.
Frisch-Wolkowitsch's stick gets to an organism in the encapsulated form and the long time remains in such look, causing the long incubatory period of a scleroma. Existence of the capsule complicates the activator macrophages and leads to formation of Mikulich's cells, specific to a scleroma, which are characterized by the large size and foamy protoplasm. Then there comes the active stage which is followed by inflammatory changes with formation of granulomas. Skleromny granulomas can have ekzofitny and endofitny growth. In the first case they block a gleam of airways, leading to violations of respiratory function. In the second case at a scleroma of a nasal cavity process can extend to skin of a nose and cause its deformations. Inflammatory process at a scleroma never occupies bone structures.
Over time skleromny granulomas undergo fibrous transformation and cicatrize. It leads to development of limited or extended cicatricial stenoses of airways in places of localization of granulomas. Transition of an inflammatory granuloma to the cicatrizing stage without its ulceration and disintegration is distinctive feature of a scleroma.
Classification of a scleroma
Depending on localization of process distinguish a scleroma of a cavity of a nose, throat, throat, trachea, bronchial tubes. The scleroma of a mouth, additional bosoms of a nose, a conjunctiva, an external and middle ear belongs to rare forms.
During a disease allocate 3 main periods: initial, active and cicatricial (regressive). As the pathological processes happening at a scleroma it can have the productive, dystrophic or mixed character. At a productive form in various departments of pneumatic ways there is a formation of granulomas and infiltrates. The dystrophic form is followed by atrophic processes in a mucous membrane with education viscous, drying up in crusts, a secret.
Development of a scleroma begins 2-3 years later from the moment of infection with Frisch-Wolkowitsch's stick. The initial stage of a disease proceeds in the form of persistently remaining general symptoms: headache, increased fatigue, weakness, drowsiness, general malaise, loss of appetite. The lowering of arterial pressure, muscular hypotonia is sometimes observed. Local changes and respectively local symptoms of a disease are absent. Long and persistent character above the listed general symptoms can be the cause for carrying out a bacteriological research on Frisch-Wolkowitsch's stick.
The clinic of the active period of a scleroma is connected with local changes. It depends on their localization, prevalence and the nature of pathological changes (a productive or dystrophic form). Inflammatory process at a scleroma can have the most various area of defeat from single small infiltrates before big and widespread opukholevidny educations. However there are several distinctive symptoms of a disease: safety of integrity of a mucous membrane and lack of formation of sinekhiya in places where infiltrates of opposite walls contact.
Most often changes at a scleroma are localized in a nasal cavity. At the beginning of a disease they are shown by banal rhinitis. At a dystrophic form of patients dryness in a nose, deterioration in sense of smell and formation of crusts which have the unpleasant lusciously sweet smell different from a smell at the ozena disturbs. At a productive form various extent of violation of nasal breath takes place. The scleroma of a throat is shown by dryness and discomfortable feelings in a throat, swallowing violation. At a process arrangement in a throat there is a hoarseness of a voice, respiratory violations as a chronic stenosis of a throat. The scleroma of a trachea and bronchial tubes is followed by allocation of a dense not plentiful phlegm.
During the cicatricial period of a scleroma there is a replacement of granulomas with connecting fabric to formation of the hems narrowing a gleam of pneumatic ways. Process is followed by development of the resistant stenoses aggravating respiratory violations and at localization in a throat capable to cause its sharp stenosis. Joán can lead scarring of the granulomas located in back departments of a nasal cavity to emergence of an atresia.
Skleromny process at one patient can affect various sites of pneumatic ways. At the same time the combination of an active stage of a scleroma on one site with a cicatricial stage on another is possible. At accession of a secondary infection the scleroma can be complicated by pharyngitis, sinusitis, laryngotracheitis, laryngitis, tracheitis, otitis, bronchitis. It is long the existing breath violations in certain cases promote development of HOBL, emphysema of lungs, a pneumosclerosis, a bronkhoektatichesky disease.
Diagnostics of a scleroma
The nonspecific nature of manifestations of a scleroma in an initial stage does its early diagnostics very difficult. If the otolaryngologist managed to suspect a scleroma, then carrying out a bakposev of dabs from a nose and a throat can unambiguously clear a diagnostic picture as in an initial stage Frisch-Wolkowitsch's stick is already sowed. Early diagnostics is important for efficiency of the subsequent treatment and the forecast of a disease.
When diagnosing a scleroma without fail conduct an endoscopic research of respiratory tract for identification of all affected areas: rinoskopiya, faringoskopiya, mikrolaringoskopiya, bronkhoskopiya. In difficult diagnostic cases carrying out an endoscopic biopsy is shown. The histologic research of a biopsiyny sample finds Mikulich's cells, characteristic of a scleroma. However it is necessary to consider that in a stage of scarring neither Mikulich's cell, nor Frisch-Wolkowitsch's stick do not come to light.
In addition appoint KT and a X-ray analysis of a throat and throat which can reveal the scattered and shapeless centers of calcification, in a cicatricial stage — ossification strips. Make inspection of okolonosovy bosoms, a research of voice function, a bronchography, a X-ray analysis of lungs. At suspicion on a scleroma of other localization of the patient direct respectively to consultation of the oculist, stomatologist, dermatologist.
Scleromas depending on localization need differential diagnostics from syphilis, Wegener's granulematoz, a lymphoma, tuberculosis, hard currency, a leprosy, benign tumors of a throat, a throat and a cavity of a nose. The scleroma of an external ear needs to be distinguished from a foreign matter of an ear and external otitis, a scleroma of a middle ear — from chronic average otitis and tumors of an ear, a conjunctiva scleroma — from conjunctivitis, a mouth scleroma — from tumors of an oral cavity, an atrophic and hypertrophic gingivit.
Treatment of a scleroma
Therapy of a scleroma includes etiotropny and anti-inflammatory treatment, X-ray therapy, a buzhirovaniye of infiltrates and stenoses, surgical restoration of passability of airways.
Therapy by streptomycin and embikhiny is directed to elimination of the activator of a scleroma. Streptomycin is applied in the form of intramuscular injections twice a day, — in the form of the intravenous drop administration in solution of glucose which is carried out every other day. Lately at a productive scleroma local X-ray therapy which is carried out at distance of 30 cm with a radiation dose 100-200r for 1 session was widely used. As a rule, the course of treatment consists of 15-20 sessions. Often the X-ray therapy begun in an initial stage of a scleroma is followed by a full rassasyvaniye of skleromny granulomas and prevents their scarring. At a dystrophic form of a scleroma oil and alkaline inhalations are shown.
Buzhirovaniye and surgical treatment of a scleroma in fact are palliative methods. They are applied at the expressed respiratory violations. The operations performed at a scleroma consist in excision of granulomas and rubtsovoizmenenny fabrics. At development of a sharp stenosis of a throat the urgent trakheostomiya is made.
Forecast of a scleroma
The forecast of a disease in many respects depends on its localization, character, prevalence and on timeliness of the begun treatment. Competently carried out treatment in an initial stage of a scleroma leads to long remission of a disease and even to an absolute recovery of the patient. However difficulties of diagnostics promote that in many cases the scleroma comes to light only in the active period that worsens the forecast. In such cases the disease lasts many years and can gain widespread character. Death of the patient is possible from asphyxia, respiratory insufficiency, bronchopulmonary complications.