Scleroma of the nose

Scleroma is a chronic infectious disease of the respiratory tract, characterized by the appearance of dense infiltrates in their mucous membranes, a slow progressive course, the appearance in the final stage of disfiguring scars deforming and stenosing the affected anatomical formations. The causative agent of the disease is the wand of Volkovitch - Frish. The disease is not very contagious, differs in the epidemic outbreaks of Belarus, Ukraine, countries of eastern and central Europe. The incubation period with the rhinoscleroma is unknown. The disease usually develops gradually, lasts for years, sometimes for decades. Exacerbation of the process is observed during pregnancy. Women are sick more often than men and children. The disease often begins at the age of 11-30 years. Most of the patients with rhinoscleroma are rural residents.

Scleroma is known since the Middle Ages. She was considered a special form of syphilis, tuberculosis, found in her resemblance to sarcoma or cancer, and only in the second half of the XIX century. It began to be studied as a separate nosological form. One of the first clonal pictures of the scleroma was described in 1858 by VA Karavaev (1911-1892) - an outstanding Russian and Ukrainian surgeon, ophthalmologist and rhinologist, one of the founders of ophthalmology and rhinoplasty. In 1870 F.Gebra - an outstanding Austrian dermatologist, head of the Austrian dermatological school in the XIXth century - suggested calling this disease a rhinosclerosis, since the 10 cases of this disease described by him concerned the defeat of the external nose. NMVolkovich in 1888 gave this disease a new name - scleroma of the respiratory tract, so it affects not only the nose, but the entire respiratory tract, up to the individual bronchi. J.Mikulicz in 1876 found in scleral infiltrates and described peculiar foamy (light) cells, called Mikulich cells. In 1876 the Austrian bacteriologist A.Frish discovered the causative agent of the scleroma with a gram-negative encapsulated rod, whose pure culture, after a few years, identified and described its morphological features NMVolkovich.

[1], [2], [3], [4], [5]

Pathological anatomy

In the development of a sclerotic infiltrate, 4 stages are distinguished.

• Stage I is characterized by a thickening of the nasal mucosa (or other localization in the upper respiratory tract) and the formation of brown-red or dark red infiltrates, which initially have a mild-elastic consistency, subsequently - dense cartilaginous consistency, are not prone to decay. At this stage, the infiltrate contains inflammatory cells (lymphocytes, plasmocytes, histiocytes and neutrophils). Among these shaped elements, larger (20-50 μm), vacuolated cells with an eccentrically located nucleus and protoplasm, rich in chromatin (Mikulich cells), begin to appear.
• In the II stage, histiocytic infiltration begins to predominate, the process of transformation of histiocytes into Mikulich cells develops. In these cells and around them, a large number of Wolkowicz-Frisch sticks and peculiar Russelean bodies, consisting of hyaline, 30-40 μm in size, are found.
• In the III stage there is a decrease in the number of histiocytes, an increase in the number of Mikulich cells, connective tissue elements and the formation of collagen fibers.
• At this stage, the scarring of sclerous infiltrates begins, which reaches its apogee in stage IV, during which the Mikulich cells and histiocytes disappear and are replaced by collagen fibers, the number of which increases with the formation of dense scar tissue. This process leads to a persistent narrowing of the lumen of the airways (partial or complete obstruction of the nasal passages, with other localization in the airways - to stenosis of the larynx, trachea and bronchi.

As a rule, scleral foci begin to develop in the nasal mucosa. Further spreading can occur in ascending or descending direction, affecting the nose, lips, sometimes the corners of the mouth, tongue, nasopharynx, soft and hard palate, upper respiratory tract, rarely - tear ducts, auditory tubes, middle ear and conjunctiva eyes .

Nasal Sclerosis Symptoms

In the initial stage, there are no significant complaints. The pathoanatomical changes that arise in the nasal mucosa do not cause pain, proceed without general reactions. The mucous membrane of the nose is a picture of banal chronic catarrhal inflammation. The development of the inflammatory process causes the occurrence of itching in the nose, frequent sneezing, mucous or mucopurulent discharge. The mucous membrane of the nose has a pale pink color. At this stage, the disease is often masked by allergic manifestations, but the antiallergic drugs used do not stop the development of the disease, and after a few years atrophy of the nasal mucosa and nasal concha is developed, resulting in nasal passages widening. Then in the nasal cavity there are crusts with an unpleasant, specific "sweet" odor, different from ozonous and syphilitic. Nasal bleeding increases, and hyposmia develops. At this stage, the rhinosclerosis is often mixed with the oaso, but with a more careful examination with a microscope on the nasal mucosa, small thickening of the mucosa can be seen in the form of papillae either in the anterior parts of the nose or in the region of the khohan. Most often, these changes occur in places of physiological narrowing of the upper respiratory tract. The period of the height of the disease is characterized by the appearance and rapid increase of scleral infiltrates, which lead to difficulty and then to a complete shutdown of nasal breathing, reducing the severity of the sense of smell to complete anosmia.

Depending on the localization of the process, the rhinoscleroma can acquire various visual aspects. Occasionally there are galloping forms of scleroma, sometimes with spreading to the bones of the nose, hard palate, alveolar process. The development of scleral infiltrates at the level of the nasal valve leads to the obstruction of the nose, and the defeat of the external nose sometimes proceeds according to the type of rhinophymus scleroma. Other anatomical lesions are rarely affected. The formation of kulis-like strands in the nasopharynx sometimes leads to an almost complete dissociation with the pharynx. Spreading on the soft palate, the process leads to its wrinkling and deformation, which makes it difficult to swallow, especially liquid food, a violation of phonation.

The general condition of the patient with sclera remains satisfactory and is disrupted only if breathing is difficult due to the onset of chronic general hypoxia. In this case, patients often violated the main and nitrogen metabolism, develops hypochromia anemia, lymphocytosis, eosinophilia, in some cases monocytopenia is observed. ESR is constantly improved.

Evolution and complications

The disease develops very slowly, for a number of years and even decades. The cases of spontaneous convalescence are unknown, while the application of even the most active and modern treatment does not guarantee a complete cure; speech can go only about temporary remission or suspension of the development of the disease. The sclerotic process can spread either along the length, or by making "jumps" through healthy parts of the mucous membrane, hitting individual segments from the nose to the koan, nasopharynx, auditory tubes, pharynx, larynx, trachea and bronchi. The paranasal sinuses are rarely affected.

Of complications, the most urgent obstruction of the airways, chronic hypoxia, pneumonia, pulmonary tuberculosis. In these cases, patients reach extreme degrees of exhaustion and die either from irreversible metabolic changes or from asphyxia (even with a timely tracheotomy) if the process caused obstruction of the trachea and bronchi.

[6], [7], [8], [9], [10], [11]

Diagnosis of Nasal Sclerosis

Diagnosis is difficult in the initial stage of the disease, since the emerging inflammatory phenomena in the nose are very similar to those in the banal catarrhal rhinitis. However, in all cases of a prolonged "runny nose" accompanied by even subtle productive processes in the mucous membrane, a specific odor from the nose, taking into account the epidemics, the presence of a rhinoscleroma should be suspected. Further examination of the patient should be aimed at identifying the disease. However, it should be borne in mind that a laboratory test does not always give a positive result, but even in the absence of the latter can not be excluded scleroma. Diagnosis is facilitated in the stage of pronounced clinical and morphological manifestations. To determine the diagnosis, endoscopy of the upper respiratory tract, biopsy, radiography, agglutination of the free-capsular strains of the Frish-Volkovich bacillus, skin allergic reactions, possibly even the binding reaction of the complement of the serum of the patient and the serum of rabbits immunized with a Frish-Volkovitch stick are important. According to several authors, the positive Borde-Gangu reaction is the most constant in sclera, however, it can not be considered specific for this disease. Crops of sclerotic material (nasal discharge, crushed biopsy samples) on agar-agar allow 80-90% of the cases to isolate the culture of the Frisch-Volkovich rod. When microscopic examination of scleral tissues, although rare, giant Gwucolized Mikulich cells, Roussel fuchsinophilic bodies and numerous plasma cells can be detected.

[12], [13], [14]

Differential diagnostics

Rinoskleroma, like the scleroma of any other localization, at different stages of development may have some similarities with various diseases. In the debut stage, it is differentiated from catarrhal or simple atrophic rhinitis, oes, syphilitic lesions. Cases of combined diseases of ozona and scleroma were described . In the mature stage, scleromatous infiltrates can be taken as manifestations of any disease characterized by the appearance of infiltrates and tumors, including tuberculosis, syphilis, leprosy, benign and malignant tumors, and others.

[15], [16], [17], [18], [19], [20]

Nasal scleroma treatment

Offered during the XIX-XX centuries. Numerous methods of treatment did not give practically no positive results. Among these methods, mention should be made of biological methods (vaccine therapy, sclerosis tissue autoimmunity, blood irradiated blood transfusion), chemical (iodine preparations, carbolic and chromic acid, zinc chloride, neo-salvarsan, quinine and bismuth preparations, etc.), physical (diathermocoagulation, radiotherapy ), mechanical (bougie), surgical (removal of cicatricial stenoses, plastic surgery, tracheotomy). Currently, relatively favorable results are obtained with streptomycin treatment (topically in the form of ointments and aerosols, parenterally with long courses but 2-3 months and X-ray therapy, and auromycin, terramycin and intravenous 5% PASK solution with encouraging results. Sclera is not manifested.

General treatment is prescribed in connection with the violation of metabolic processes: calcium preparations, vitamin therapy, drugs that normalize CBS, complete carbohydrate and protein nutrition.

The prognosis for complete recovery in far-reaching cases is unfavorable.