Rhinoscleroma

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Rhinoscleroma Classification and external resources
ICD-9	040.1
DiseasesDB	31327
eMedicine	derm/831
MeSH	C01.252.400.310.503.650

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Rhinoscleroma is a chronic granulomatous disease of the upper respiratory tract.

1 Synonyms
2 Incidence
3 Causes
4 Symptoms
5 Diagnosis
6 Historical background
7 Treatment
8 Prognosis
9 See also
10 References

[edit] Synonyms

Mikulicz disease

[edit] Incidence

More females than males are affected. Patients are usually 10 to 30 years of age. Mostly seen in Africa and central America. Less in United States. common in tropical countries.

[edit] Causes

It is caused by Klebsiella rhinoscleromatis, a gram-negative, encapsulated, nonmotile, diplobacillus and member of the Enterobacteriaceae family. It is contracted directly by droplets or by contamination of material that is subsequently inhaled.

[edit] Symptoms

It most commonly affects the nasal cavity; however, it can affect the nasopharynx, larynx, trachea, and bronchi. 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 deformaty, and destruction of the nasal cartilage are also noted (Heba 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.

[edit] Diagnosis

A positive culture in MacConkey agar is dagnostic, 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).

[edit] Historical background

Photogravure of rhinoscleroma from Wolkowitsch.

First described by Hans von Hebra (1847-1902) in a paper titled Ueber ein eigenthümliches Neugebilde an der Nase; Rhinosclerom; nebst histologischem Befunde vom Dr. M. Kohn, published in the January 1870 issue of the Wiener Medizinische Wochenschrift (vol. 20, pp. 1-5). Hans was the son of the Czech born dermatologist Ferdinand Ritter von Hebra (1816-1880), founder of the New Vienna School of Dermatology.

M. Kohn, who did the histology for the paper, is the name by birth of Moritz Kaposi (1837-1902).