Rhinoscleroma, is a chronic

granulomatous A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Such substances include infectiou ...

bacterial disease of the nose that can sometimes infect the

upper respiratory tract The respiratory tract is the subdivision of the respiratory system involved with the process of respiration in mammals. The respiratory tract is lined with respiratory epithelium as respiratory mucosa. Air is breathed in through the nose to t ...

.Palmer & Reeder. 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 The pharynx (plural: pharynges) is the part of the throat behind the mouth and nasal cavity, and above the oesophagus and trachea (the tubes going down to the stomach and the lungs). It is found in vertebrates and invertebrates, though its struct ...

, larynx,

trachea The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air- breathing animals with lungs. The trachea extends from the ...

, and

bronchi A bronchus is a passage or airway in the lower respiratory tract that conducts air into the lungs. The first or primary bronchi pronounced (BRAN-KAI) to branch from the trachea at the carina are the right main bronchus and the left main bronchus. ...

. 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 North Africa, South Asia and Central America, less common in the United States.

Signs and symptoms

Rhinoscleroma has been divided into 3 stages: catarrhal/atrophic, granulomatous, and sclerotic stages. The catarrhal stage begins with a nonspecific

rhinitis Rhinitis, also known as coryza, is irritation and inflammation of the mucous membrane inside the nose. Common symptoms are a stuffy nose, runny nose, sneezing, and post-nasal drip. The inflammation is caused by viruses, bacteria, irrita ...

, which progresses into purulent, fetid

rhinorrhea Rhinorrhea, rhinorrhoea, or informally runny nose is the free discharge of a thin mucus fluid from the nose; it is a common condition. It is a common symptom of allergies ( hay fever) or certain viral infections, such as the common cold or CO ...

, 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 The palatine uvula, usually referred to as simply the uvula, is a conic projection from the back edge of the middle of the soft palate, composed of connective tissue containing a number of racemose glands, and some muscular fibers. It also conta ...

dysphonia A hoarse voice, also known as dysphonia or hoarseness, is when the voice involuntarily sounds breathy, raspy, or strained, or is softer in volume or lower in pitch. A hoarse voice, can be associated with a feeling of unease or scratchiness in the ...

, and various degrees of airway obstruction. The fibrotic stage is characterized by sclerosis and

fibrosis Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perma ...

. Lymphadenitis is absent.

Causes

It is caused by Klebsiella rhinoscleromatis—subspecies of ''

Klebsiella pneumoniae ''Klebsiella pneumoniae'' is a Gram-negative, non-motile, encapsulated, lactose- fermenting, facultative anaerobic, rod-shaped bacterium. It appears as a mucoid lactose fermenter on MacConkey agar. Although found in the normal flora of the mo ...

''— a

gram-negative Gram-negative bacteria are bacteria that do not retain the crystal violet stain used in the Gram staining method of bacterial differentiation. They are characterized by their cell envelopes, which are composed of a thin peptidoglycan cell wa ...

, encapsulated, nonmotile, rod-shaped

bacillus ''Bacillus'' (Latin "stick") is a genus of Gram-positive, rod-shaped bacteria, a member of the phylum '' Bacillota'', with 266 named species. The term is also used to describe the shape (rod) of other so-shaped bacteria; and the plural ''Bacill ...

(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. It is contracted directly by droplets or by contamination of material that is subsequently inhaled.

Diagnosis

A positive culture in

MacConkey agar MacConkey agar is a selective and differential culture medium for bacteria. It is designed to selectively isolate Gram-negative and enteric (normally found in the intestinal tract) bacteria and differentiate them based on lactose fermentation. ...

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 plasma cells with birefringent inclusions, Russell bodies, pseudoepitheliomatous hyperplasia, and groups of large vacuolated histiocytes containing ''Klebsiella rhinoscleromatis'' (Mikulicz cells).

Treatment

It is not lethal in nature and is responsive to

tetracycline Tetracycline, sold under various brand names, is an oral antibiotic in the tetracyclines family of medications, used to treat a number of infections, including acne, cholera, brucellosis, plague, malaria, and syphilis. Common side effects in ...

ciprofloxacin Ciprofloxacin is a fluoroquinolone antibiotic used to treat a number of bacterial infections. This includes bone and joint infections, intra abdominal infections, certain types of infectious diarrhea, respiratory tract infections, skin inf ...

. Surgical treatment include rhinoplasty. However, if left untreated the disease can lead to

sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...

bleeding Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, vag ...

, 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. Hans von Hebra was the son of Czech born

dermatologist Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medica ...

Ferdinand Ritter von Hebra Ferdinand Karl Franz Schwarzmann, Ritter von Hebra (7 September 1816, in Brno, Moravia – 5 August 1880 in Vienna, Austria-Hungary) was an Austrian physician and dermatologist known as the founder of the New Vienna School of Dermatology, an ...

(1816–1880), founder of the New Vienna School of Dermatology. He was assisted by M. Kohn who provided much of the

histology Histology, also known as microscopic anatomy or microanatomy, is the branch of biology which studies the microscopic anatomy of biological tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures vi ...

for the paper. M. Kohn is the birth name of

Moritz Kaposi Moritz Kaposi ( hu, Kaposi Mór, ; 23 October 1837 in Kaposvár, Hungary – 6 March 1902 in Vienna, Austria-Hungary) was a Hungarian physician and dermatologist who discovered the skin tumor that received his name (Kaposi's sarcoma). Biography ...

(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.

Terminology

Hebra nose. Scleroma. Fr: Sclérome. Sp: Rinoscleroma. Ger: Sklerom. Nasen-Rachenrauminduration. Archaic terms include: Syphilis of the nose. Nasal leprosy. Scleroma neonatorum. Scleroma respiratorum. Scrofulous lupus.DiBartolomeo (''et alia'').

Notes

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 ().

External links

DiBartolomeo, ''Scleroma of the nose and pharynx.''
{{Bacterial cutaneous infections Bacterium-related cutaneous conditions