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Rhinoscleroma

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 09, 2016
 

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 Medscape article Klebsiella Infections may be of interest.

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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]

A murine model has been described delineating Mikulicz cells as interleukin 10–dependent derivatives of inflammatory monocytes.[4]

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Epidemiology

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.[5] Five percent of all cases occur in Africa.[6] However, with current trends in migration, the incidence of rhinoscleroma may be on the rise.[7] 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.[8] It is rare in Western Europe.[9]

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.

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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.[10] 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.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Egle Goriniene, MD Staff Physician, Department of Infectious Diseases, New Jersey Medical School

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

References
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