Rhinoscleroma, is a chronic granulomatous bacterial disease of the nose that can sometimes infect the upper respiratory tract.[1] 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.
Contents
1Signs and symptoms
2Causes
3Diagnosis
4Prognosis and treatment
5History
6Terminology
7See also
8Notes
9References
10External links
Signs and 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. Nose bleeds, 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 is absent.
Causes
It is caused by Klebsiella rhinoscleromatis—subspecies of
Klebsiella pneumoniae— a gram-negative, encapsulated, nonmotile, rod-shaped bacillus (diplobacillus), member of the family Enterobacteriaceae. It is sometimes referred to as the "Frisch bacillus," named for Anton von Frisch who identified the organism in 1882.[2] It is contracted directly by droplets or by contamination of material that is subsequently inhaled.[1]
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 and treatment
It is not lethal in nature and is responsive to tetracycline or ciprofloxacin. Surgical treatment include rhinoplasty. However, if left untreated the disease can lead to sepsis, bleeding, or other chronic conditions that can be fatal.
History
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.[3][4] 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.[5]
Archaic terms include: Syphilis of the nose. Nasal leprosy. Scleroma neonatorum. Scleroma respititorum. Scrofulous lupus.[6]
See also
Erythrasma
List of cutaneous conditions
List of inclusion bodies that aid in diagnosis of cutaneous conditions
Notes
^ abPalmer & Reeder.
^DiBartolomeo. Page 14.
^Hebra.
^Garrison & Morton (GM 3277).
^Frisch.
^DiBartolomeo (et alia).
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).
The Pulsatile Gonadorelin Pump Induces Earlier Spermatogenesis Than Cyclical Gonadotropin Therapy in Congenital Hypogonadotropic Hypogonadism Men.
Zhang L, Cai K, Wang Y, Ji W, Cheng Z, Chen G, Liao Z.
American journal of men's health. ;13(1)1557988318818280.
The objective of this study was to compare the effect of pulsatile gonadorelin pump (PGP) and cyclical gonadotropin (human chorionic gonadotropin [HCG]/human menopausal gonadotropin [HMG]) therapy (CGT) on spermatogenesis in congenital hypogonadotropic hypogonadism (CHH) men. Twenty-eight azoospermi
Archives of pathology & laboratory medicine. 2018 Dec;142(12)1533-1536.
Rhinoscleroma is a rare, chronic, infectious granulomatous process involving the upper respiratory tract caused by gram-negative bacilli, Klebsiella rhinoscleromatis. The site most commonly affected is the nasopharynx; however, lesions in various other locations have been described. To review the li
Report of a case of giant rhinoscleroma: CT and MRI.
Ibrahim D, Fayed A.
BJR case reports. 2018 Dec;4(4)20180027.
Rhinoscleroma is a chronic granulomatous bacterial infection caused by the gram-negative bacillus . It predominately involves the nasal cavities but it can also involve the rest of the upper respiratory tract. Hypertrophic stage of rhinoscleroma may cause large tumor masses which could mimic neoplas
A scleroma is a hardened patch of tissue in the skin or mucous membranes. It most often forms in the head and neck. The nose is the most common location for scleromas, but they can also form in the throat
In literature review of respiratory tract scleroma, a case of scleroma presenting as an upper tracheal mass causing stridor and respiratory distress was described, The patient was managed with urgent resection and an eight week ...