Rhinoscleroma. A disease of the nose and nasopharynx characterized by a hard swelling of the nasal vestibule, nasal septum, and upper lip.
Summary
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- 1 Etiology
- 2 Pathological anatomy
- 3 Clinical manifestations
- 4 Diagnosis
- 5 Treatment
- 6 Sources
Etiology
The disease is attributed to Klebsiella rhinoscleromatis (Von Frisch bacillus), a gram-negative rod of the coliform group, although this extreme has not been demonstrated. The germ is always present in lesions , which is why it has also been found in normal noses.
Other etiological factors may be admitted to remove the geographic distribution and sporadic invasion of the germ. The disease is barely contagious. Most of the patients belong to groups of low socioeconomic status.
Pathological anatomy
The disease affects only the soft parts, in particular the mucosa and submucosa of the upper respiratory system. The anatomical picture goes through three phases:
- Exudation, associated with increased colorless mucous discharge and crusting, especially in the nose.
- Proliferation, characterized by the appearance of masses of granulomatous tissue in the affected areas.
- Scarring, which produces retractable scars and deformity.
The critical microscopic picture is characterized by an infiltrate of plasma and mononuclear cells, Mikulicz cells, Rusell bodies, connective tissue and proliferation, vessel hyalinization, epithelial atrophy or hypertrophy and squamous metaplasia , the Mikulicz cell is admitted as characteristic, which is a cell that contains gram-negative germs, however, these findings also take place in other diseases and are not entirely specific. Also in the final stages of fibrosis , Mikulicz cells and plasma cells are scarce, and the picture is typical of non-specific chronic inflammation.
The infraglottic region of the larynx is specifically affected, which is the seat of a large granulomatous mass or diffuse infiltration. Nodular infiltrations can affect the interearytenoid regions and the ariepligotic folds. There are no ulcerations.
Clinical manifestations
The hoarseness and cough are indicative of laryngeal disease, and may even be the initial signs in patients with nasal lesions. Stridor and dyspnea secondary to proliferative tissue obstruction of the larynx are late symptoms. In general, nasal obstruction or deformity of the nose and upper lip is seen when the patient with hoarseness is first observed.
In the early stages, the mucosa of the interarhenoid zone, the ariepiglottic folds, the laryngeal surface of the epiglottis, and the infraglottic areas are hyperemic and have a granular state. There are crusts and a purulent exudate with a yellowish coloration. Later, a reddish nodular infiltrate is observed, especially in the infraglottic region.
In some cases there is a marked tissue hypertrophy that leads to the phonation of polypoid lesions. In the final stages, the nodules acquire a firm and pale appearance and a cartilaginous consistency.
Diagnosis
The diagnosis is made by isolating Klebsiella rhinoscleromatis from a piece of tissue. The pathological aspect in the profilerative phase is also pathognomonic, but it is often of little relevance in the scar phase. Complement fixation tests with the patient’s serum are of doubtful value. Laryngeal lesions must be differentiated from syphilis , tuberculosis , leprosy, and neoplasms by means of laryngeal biopsy.
Treatment
Treatment with a combination of gentamicin or kanamycin with cephalothin is effective, achieving arrest and remission of proliferative lesions. The adjuvant use of corticosteroids can contribute to decrease scar deformity.
In the final phase of scar retraction, the specific therapy is of little value, and the treatment must be that of laryngeal obstruction. Surgical removal is necessary to remove obstructive polypoid masses. There is no indication for radiation therapy .
