Rhinoscleroma

Rhinoscleroma
Photogravure of rhinoscleroma from Wolkowitsch.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Klebsiella rhinoscleromatis; scleroma.

Symptoms

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.lymphadenitis are absent.

Diagnosis

Micrograph showing abundant subepithelial histiocytes in a case of rhinoscleroma. H&E stain.

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).

Prognosis & treatment

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

Hebra nose. Scleroma. Fr: Sclérome. Sp: Rinoscleroma. Ger: Sklerom. Nasen-Rachenrauminduration.

Archaic terms include: Syphilis of the nose. Nasal leprosy. Scleroma neonatorum. Scleroma respititorum. Scrofulous lupus.

See also

• Erythrasma
• List of cutaneous conditions
• List of inclusion bodies that aid in diagnosis of cutaneous conditions

References

• DiBartolomeo, Joseph R. (1976), Scleroma of the nose and pharynx. Journal: West. J. Med., vol. 124., pp. 13–17.
• 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.
• Morton, Leslie T. (1970), A medical bibliography (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).

External links

• Cabinet of Art and Medicine.
• DiBartolomeo, Scleroma of the nose and pharynx.
• Palmer and Reeder.