eMedicine Specialties > Dermatology > Bacterial Infections

Rhinoscleroma

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Egle Goriniene, MD, Staff Physician, Department of Infectious Diseases, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jul 2, 2009

Introduction

Background

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 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

The eMedicine article Klebsiella Infections may be of interest.

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. 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.

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.²

A Mexican study showed that DQA1*03011-DQB1*0301 haplotype is a strong risk factor for its development.³

Frequency

It is endemic to 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. Rhinoscleroma reportedly also is rare in Saudi Arabia and Bahrain.⁴ Five percent of all cases occur in Africa.⁵ However, with current trends in migration, the incidence of rhinoscleroma may be on the rise.⁶ 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.

Mortality/Morbidity

• 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.

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.

Clinical

History

Rhinoscleroma is rare chronic granulomatous infection that should be considered in patients from countries in which the disease is endemic if they have nasal polyps that significantly adhere to the nasal septum with relative sparing of the sinuses. Most often, the presentation is nonspecific. Because of its mundane clinical presentation resembling chronic rhinitis, it often goes unrecognized.⁷ Chronic nasal infection caused by K rhinoscleromatis is often a misdiagnosed infectious disease.⁸ Chronic rhinitis, even in developed countries, should prompt its consideration, especially because specific diagnostic tools and effective treatments are available.

• Nasal obstruction (most common complaint)
• Rhinorrhea
• Epistaxis
• Dysphagia
• Nasal deformity
• Anesthesia of the soft palate
• Difficulty breathing that progresses to stridor
• Dysphonia
• Anosmia
• In persons with pseudotumoral rhinoscleromas in the septum and in the rhinopharynx, respectively, CT scanning permitted a precise evaluation of the extent of the lesions.

Physical

The disease commonly affects the nasal cavity (95-100% of patients), but it can also affect the nasopharynx (18-43%), larynx (15-40%), trachea (12%), and bronchi (2-7%). The oral cavity, paranasal sinuses, and soft tissues of the lips and nose can be affected as well. In rare cases, rhinoscleroma spreads to the orbit.

Most often, the presentation is nonspecific.

Rhinoscleroma is divided into 3 stages: (1) the catarrhal, or atrophic, stage; (2) the granulomatous stage; and (3) the sclerotic stage.

• Catarrhal, or atrophic, stage
  • This first stage begins with a nonspecific rhinitis that evolves into purulent fetid rhinorrhea and crusting.
  • This stage can last for weeks or months.
• Granulomatous, or hypertrophic, stage
  • The nasal mucosa becomes bluish red and granular, with the formation of rubbery nodules or polyps in the nose.
  • Epistaxis occurs with nasal enlargement, deformity, and destruction of the nasal cartilage (Hebra nose).
  • The damage may progress to anosmia, anesthesia of the soft palate, enlargement of the uvula, dysphonia, and various degrees of airway obstruction.
• Lesions occur in the form of atrophic changes and granulomas, and in the fibrotic, thick, healed stage.
• The anterior-inferior part of the antrum and its medial wall are more commonly affected than other structures.
• Involvement of the maxillary antrum is suggested in scleroma, and the maxillary antrum may act as a reservoir of infection.
• The soft palate is markedly thickened at its attachment to the hard palate, which tapers off toward its free edge. This sign can help in the early diagnosis of the condition.
• Physical examination frequently reveals erythematous granular or nodular swellings covered with crusts.
• The tumorlike appearance and local spread are suggestive of malignancy. The pseudotumoral rhinoscleromas may be located in a variety of sites, including the septum and in the rhinopharynx.⁷
• Sclerotic stage
• The sclerotic stage is characterized by sclerosis and fibrosis.
• The sclerotic stage develops where the nodules are replaced by fibrous tissue leading to extensive scarring and possible stenosis.

Causes

• 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.

• Search for CME/CE on This Topic »

• Klebsiella Infections (Infectious Diseases)
• Nasopharyngeal Stenosis (Otolaryngology and Facial Plastic Surgery)
• Granulomatous Diseases of the Head and Neck (Otolaryngology and Facial Plastic Surgery)
• Glottic Stenosis (Otolaryngology and Facial Plastic Surgery)