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{{Infobox Disease |
  Name          = Rhinoscleroma |
  Image          = Wolkowitsch1.jpg |
  Caption        = Photogravure of rhinoscleroma from Wolkowitsch. |
}}
'''For patient information, click [[Rhinoscleroma (patient information)|here]]'''
'''For patient information, click [[Rhinoscleroma (patient information)|here]]'''
{{Rhinoscleroma}}
{{Rhinoscleroma}}
{{CMG}}
{{CMG}}; {{AE}} {{KS}}


{{SK}} Klebsiella rhinoscleromatis.
{{SK}} Klebsiella rhinoscleromatis; scleroma; syphilis of the nose; nasal leprosy; scleroma neonatorum; srofulous lupus.
==Overview==
'''Rhinoscleroma''', or simply '''Scleroma''', is a chronic [[granulomatous]] bacterial disease of the nose that can sometimes infect the [[upper respiratory tract]].<ref>Palmer & Reeder.</ref> It most commonly affects the nasal cavity—the nose is involved in 95-100 per cent of cases—however, it can also affect the nasopharynx, larynx, trachea, and bronchi. Slightly more females than males are affected and patients are usually 10 to 30 years of age. Rhinoscleroma is considered a tropical disease and is mostly endemic to Africa and central America, less common in the United States.  


==Causes==
==[[Rhinoscleroma overview|Overview]]==


It is caused by [[Klebsiella rhinoscleromatis]]—subspecies of
==[[Rhinoscleroma historical perspective|Historical Perspective]]==
[[Klebsiella pneumoniae]]— a gram-negative, encapsulated, nonmotile,  rod-shaped [[bacillus]] (diplobacillus), member of the [[Enterobacteriaceae]] family. It is sometimes referred to as the "Frisch bacillus," named for Anton von Frisch who identified the organism in 1882. <ref>DiBartolomeo. Page 14.</ref> It is contracted directly by droplets or by contamination of material that is subsequently inhaled.<ref>Palmer & Reeder.</ref>


==Symptoms==
==[[Rhinoscleroma pathophysiology|Pathophysiology]]==


Rhinoscleroma has been divided into 3 stages: catarrhal/atrophic, granulomatous, and sclerotic stages.  The catarrhal stage begins with a nonspecific rhinitis, which progresses into purulent, fetid rhinorrhea, and crusting, which can last for weeks or even months.  The granulomatous stage results in the development of a bluish red nasal mucosa and the development of intranasal rubbery nodules or polyps.  Epistaxis, nasal deformity, and destruction of the nasal cartilage are also noted (Hebra nose).  The damage may result in anesthesia of the soft palate, enlargement of the uvula, dysphonia, and various degrees of airway obstruction.  The fibrotic stage is characterized by sclerosis and fibrosis.
==[[Rhinoscleroma causes|Causes]]==


==Diagnosis==
==[[Rhinoscleroma differential diagnosis|Differentiating Rhinoscleroma from other Diseases]]==


A positive culture in MacConkey agar is diagnostic, but cultures are only positive in 50-60% of cases.  Diagnostic characteristics are most commonly found in the granulomatous stage and are described as being chronic inflammatory cells, [[Russell bodies]], and pseudoepitheliomatous hyperplasia, and groups of large vacuolated histiocytes containing ''Klebsiella rhinoscleromatis'' (Mikulicz cells).
==[[Rhinoscleroma epidemiology and demographics|Epidemiology and Demographics]]==


==Prognosis & treatment==
==[[Rhinoscleroma risk factors|Risk Factors]]==
It is not lethal in nature and is responsive to [[Tetracycline]] or [[Ciprofloxacin]]. However, if left untreated the disease can lead to sepsis, hemorrhage or other chronic conditions that can be fatal.


==Synonyms==
==[[Rhinoscleroma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
Hebra nose. Scleroma.  Fr: Sclérome. Sp: Rinoscleroma.  Ger: Sklerom. Nasen-Rachenrauminduration.
<br />


Archaic terms include: Syphilis of the nose. Nasal leprosy. Scleroma neonatorum. Scleroma respititorum. Scrofulous lupus. <ref>DiBartolomeo (''et alia'').</ref>
==Diagnosis==


==History==
[[Rhinoscleroma history and symptoms|History and Symptoms]] | [[Rhinoscleroma physical examination|Physical Examination]] | [[Rhinoscleroma laboratory findings|Laboratory Findings]] | [[Rhinoscleroma imaging findings|Imaging Findings]] | [[Rhinoscleroma other diagnostic studies|Other Diagnostic Studies]]


[[Image:Wolkowitsch1.jpg|right|thumb|Photogravure of rhinoscleroma from Wolkowitsch.]]
==Treatment==


Hans von Hebra (1847-1902)  wrote the classical description of the disease  in a paper published in the January 1870 issue of the Wiener Medizinische Wochenschrift.<ref>Hebra.</ref><ref>Garrison & Morton (GM 3277).</ref> Hans von Hebra was the son of  Czech born dermatologist [[Ferdinand Ritter von Hebra]] (1816-1880), founder of the New Vienna School of Dermatology. He was assisted by  M. Kohn who provided much of the histology for the paper. M. Kohn is the birth name of [[Moritz Kaposi]] (1837-1902).  In 1876, Mikulicz contributed to the microscopic histology. In 1882, Anton Von Frisch (1849-1917) discovered the gram-negative bacillus which causes the disease.<ref>Frisch.</ref>
[[Rhinoscleroma medical therapy|Medical Therapy]] | [[Rhinoscleroma surgery|Surgery]] | [[Rhinoscleroma prevention|Prevention]] | [[Rhinoscleroma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Rhinoscleroma future or investigational therapies|Future or Investigational Therapies]]


==Notes==  
==Case Studies==
{{Reflist}}


==References==  
[[Rhinoscleroma case study one|Case #1]]
== Related Chapters ==
* [[Erythrasma]]


* DiBartolomeo, Joseph R. (1976), ''Scleroma of the nose and pharynx.'' Journal: West. J. Med., vol. 124., pp. 13-17.
==External links==
* Frisch, Anton von (1882), ''Zur Aetiologie des Rhinoskleroms.'' Journal: Wiener Medizinische Wochenschrift; vol. 32, pp. 969-972.
 
* Hebra, Hans von (1870), ''Ueber ein eigenthümliches Neugebilde an der Nase; Rhinosclerom; nebst histologischem Befunde vom Dr. M. Kohn.'' Journal: Wiener Medizinische Wochenschrift; vol. 20, pp. 1-5.
* [http://www.pubmedcentral.nih.gov/pagerender.fcgi?artid=1129960&pageindex=1 DiBartolomeo, ''Scleroma of the nose and pharynx.'']
* Morton, Leslie T. (1970), ''A medical bibliography (Fielding H. Garrison and Morton).'' Philadelphia & Toronto: J. B. Lippincott Company,  p. 388.
*  Palmer, P. E. S. & Reeder, M. M. (2000), ''The imaging of tropical diseases.'' Heidelberg: Springer Verlach; vols. 1 & 2 (ISBN 3-540-66219-7).


{{Bacterial diseases}}
{{Bacterial diseases}}


[[Category:Bacterium-related cutaneous conditions]]




[[lt:Rinoskleroma]]
[[pl:Twardziel (choroba)]]
[[pl:Twardziel (choroba)]]
[[Category:Infectious disease]]
[[Category:Overview complete]]
{{WH}}
{{WS}}

Latest revision as of 18:37, 18 September 2017

Rhinoscleroma
Photogravure of rhinoscleroma from Wolkowitsch.

For patient information, click here

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Synonyms and keywords: Klebsiella rhinoscleromatis; scleroma; syphilis of the nose; nasal leprosy; scleroma neonatorum; srofulous lupus.

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Rhinoscleroma from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

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