Rhinoscleroma

Updated: Jun 27, 2023
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
  • Print
Overview

Practice Essentials

Rhinoscleroma is a chronic granulomatous condition of the nose and other structures of the upper respiratory tract. Rhinoscleroma is a result of infection by the bacterium Klebsiella rhinoscleromatis.

The Medscape Drugs & Diseases article Klebsiella Infections may be of interest.

Signs and symptoms

Also see Presentation.

The condition often presents as an intra-nasal obstruction with nonspecific signs. Nodules are usually small initially. The disease should be considered in endemic areas.

Diagnostics

Also see Workup.

Culture results for rhinoscleroma are unreliable. Biopsy analysis remains a reliable diagnostic method.

MRI should be undertaken when rhinoscleroma is suspected.

Management

Also see Treatment.

Long-term antimicrobial therapy is the medical approach of choice to rhinoscleroma. Surgical intervention is indicated in cases that feature nasal obstruction.

Next:

Background

The Polish surgeon Johann von Mikulich in Wroclaw described the histologic features in 1877; von Frisch identified the organism in 1882. In 1932, Belinov proposed the use of the term scleroma respiratorium because the pathologic process in rhinosclerosis may involve not only the upper airways but also the lower airways. In 1961, Steffen and Smith demonstrated that K rhinoscleromatis conformed to Koch's postulates and that it was an etiologic factor in the inflammatory changes typical of scleroma. The occurrence of familial disease suggests that genetic control of the host response to K rhinoscleromatis may be an important factor in endemic areas. [1] Rhinoscleroma is a chronic infectious disease of underdeveloped countries. [2]

Previous
Next:

Pathophysiology

Rhinoscleroma is contracted by means of the direct inhalation of droplets or contaminated material. The disease probably begins in areas of epithelial transition such as the vestibule of the nose, the subglottic area of the larynx, or the area between the nasopharynx and oropharynx. Cellular immunity is impaired in patients with rhinoscleroma; however, their humoral immunity is preserved.

The CD4/CD8 cell ratio in the lesion is altered with decreased levels of CD4 lymphocytes; this change possibly induces a diminished T-cell response. Macrophages are not fully activated. Mucopolysaccharides in the bacterial capsule probably contribute to the inhibition of phagocytosis. The type K3 capsule of K rhinoscleromatis serves as a virulence factor. [3] Otherwise, patients are immunocompetent in every regard except for the ineffective phagocytosis of the organism by the Mikulicz cells.

Rhinoscleroma usually affects the nasal cavity, but lesions associated with rhinoscleroma may also affect the larynx; nasopharynx; oral cavity; paranasal sinuses; or soft tissues of the lips, nose, trachea, and bronchi. Rhinoscleroma may be evident as a tumor extruded from the mucosal lining of the upper lip. [4] It may also appear on the penis, where its presence may suggest a penile cancer. [5]  

Although it is usually caused by K pneumoniae subsp rhinoscleromatis, K pneumoniae subsp ozaenae was isolated from the pharynx of a woman with laryngeal scleroma. [6]

A Mexican study showed that DQA1*03011-DQB1*0301 haplotype is a strong risk factor for its development. [7]

A murine model has been described delineating Mikulicz cells as interleukin 10–dependent derivatives of inflammatory monocytes. [8]

Previous
Next:

Etiology

Rhinoscleroma is caused by the gram-negative coccobacillus K rhinoscleromatis. Although crowded conditions, poor hygiene, and poor nutrition appear to be necessary for transmission of the infectious agent, the actual pathogenesis of infection remains elusive.

Previous
Next:

Epidemiology

Frequency

It is endemic to poor regions of Africa (Egypt, tropical areas), Southeast Asia, Mexico, Central and South America, and Central and Eastern Europe, but it has been infrequent in the United States. [9] Rhinoscleroma reportedly also is rare in Saudi Arabia and Bahrain. [10] Five percent of all cases occur in Africa. [11] However, with current trends in migration, the incidence of rhinoscleroma may be on the rise. [12] The incidence of rhinoscleroma appears to be increasing in the United States. Rare sporadic cases occur in the United States, usually in immigrant populations arriving from the countries in which the disease is endemic. Rhinoscleroma is endemic to areas of Africa (Egypt, tropical areas), Southeast Asia, Mexico, Central and South America, and Central and Eastern Europe, with an increased incidence in Spain possibly due to new immigrants from endemic regions. [13] It is rare in Western Europe. [14]  In Egypt, its incidence seems to be decreasing, with clinical and histopathological patterns in less advanced forms, presumably owing to socioeconomic and treatment advances. [15]

Race

Patients of all races can be affected.

Sex

Rhinoscleroma tends to affect females somewhat more often than it does males.

Age

Typically, rhinoscleroma appears in patients aged 10-30 years.

Previous
Next:

Prognosis

The course is usually chronic. Relapses can occur. Despite antibiotic and surgical therapy, an incidence of recurrence in one series reached up to 25% within 10 years. [16] Rhinoscleroma is rarely lethal, unless it causes airway obstruction. The diagnosis may elude the clinician for years, and this delay can substantially increase the rate or severity of resultant morbidity.

Previous