Rhinoscleroma

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Rhinoscleroma
Wolkowitsch1.jpg
Photogravure of rhinoscleroma from Wolkowitsch.
Specialty Infectious disease   Blue pencil.svg

Rhinoscleroma, or simply scleroma, 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.

Larynx voice box, an organ in the neck of amphibians, reptiles, and mammals

The larynx, commonly called the voice box, is an organ in the top of the neck of tetrapods involved in breathing, producing sound, and protecting the trachea against food aspiration. The larynx houses the vocal folds, and manipulates pitch and volume, which is essential for phonation. It is situated just below where the tract of the pharynx splits into the trachea and the esophagus. The word larynx comes from a similar Ancient Greek word.

Trachea cartilaginous tube that connects the pharynx and larynx to the lungs

The trachea, colloquially called the windpipe, is a cartilaginous tube that connects the pharynx and larynx to the lungs, allowing the passage of air, and so is present in almost all air-breathing animals with lungs. The trachea extends from the larynx and branches into the two primary bronchi. At the top of the trachea the cricoid cartilage attaches it to the larynx. This is the only complete tracheal ring, the others being incomplete rings of reinforcing cartilage. The trachealis muscle joins the ends of the rings and these are joined vertically by bands of fibrous connective tissue – the annular ligaments of trachea. The epiglottis closes the opening to the larynx during swallowing.

Contents

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.

Rhinitis irritation and inflammation of the mucous membrane inside the nose

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.

Rhinorrhea type of medical symptom where the nasal cavity is filled with fluid mucus

Rhinorrhea or rhinorrhoea is a condition where the nasal cavity is filled with a significant amount of mucus fluid. The condition, commonly known as a runny nose, occurs relatively frequently. Rhinorrhea is a common symptom of allergies or certain viral infections, such as the common cold. It can be a side effect of crying, exposure to cold temperatures, cocaine abuse or withdrawal, such as from opioids like methadone. Treatment for rhinorrhea is not usually necessary, but there are a number of medical treatments and preventive techniques available.

Anesthesia state of medically controlled temporary loss of sensation or awareness

Anesthesia or anaesthesia is a state of controlled, temporary loss of sensation or awareness that is induced for medical purposes. It may include analgesia, paralysis, amnesia, or unconsciousness. A patient under the effects of anesthetic drugs is referred to as being anesthetized.

Causes

It is caused by Klebsiella rhinoscleromatis —subspecies of 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. [2] It is contracted directly by droplets or by contamination of material that is subsequently inhaled. [1]

<i>Klebsiella pneumoniae</i> species of bacterium

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.

<i>Bacillus</i> genus of bacteria

Bacillus is a genus of Gram-positive, rod-shaped bacteria, a member of the phylum Firmicutes, with 266 named species. The term is also used to describe the shape (rod) of certain bacteria; and the plural Bacilli is the name of the class of bacteria to which this genus belongs. Bacillus species can be either obligate aerobes: oxygen dependent; or facultative anaerobes: having the ability to be anaerobic in the absence of oxygen. Cultured Bacillus species test positive for the enzyme catalase if oxygen has been used or is present.

Enterobacteriaceae family of bacteria

The Enterobacteriaceae are a large family of Gram-negative bacteria. This family is the only representative in the order Enterobacteriales of the class Gammaproteobacteria in the phylum Proteobacteria.

Diagnosis

Micrograph showing abundant subepithelial histiocytes in a case of rhinoscleroma. H&E stain. Rhinoscleroma - very high mag.jpg
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).

MacConkey agar culture medium used in microbiology

MacConkey agar is an indicator, a selective and differential culture medium for bacteria designed to selectively isolate Gram-negative and enteric bacilli and differentiate them based on lactose fermentation. The crystal violet and bile salts inhibit the growth of Gram-positive organisms which allows for the selection and isolation of gram-negative bacteria. Enteric bacteria that have the ability to ferment lactose can be detected using the carbohydrate lactose, and the pH indicator neutral red.

Russell bodies

Russell bodies are eosinophilic, large, homogeneous immunoglobulin-containing inclusions usually found in a plasma cell undergoing excessive synthesis of immunoglobulin; the Russell body is characteristic of the distended endoplasmic reticulum. They are found in the peripheral areas of tumors. Russell bodies are thought to have originated as an SOS compartment, where abnormal proteins that are not secreted, but have fled intracellular degradation, can gather without blocking normal secretory pathways. The excess immunoglobulin builds up and forms intracytoplasmic globules, which is thought to be a result of insufficient protein transport within the cell. This causes the proteins to neither be degraded or secreted and stay stored in dilated cisternae. In 1949, Pearse discovered that Russell bodies also contain mucoproteins that are secreted by plasma cells. Russell bodies are not tissue specific; during research they were induced in rat glioma cells. Russell bodies were found to have positive reactions to PAS stain, CD 38 and CD 138 stains. Plasma cells that contain Russell bodies and are stained with H&E stain are found to be autofluorescent, while those without Russell bodies are not autofluorescent. Russell bodies tend to be found in places with chronic inflammation. Multiple aggregrates of Russell bodies create what are known as Mott 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.

Tetracycline chemical compound

Tetracycline, sold under the brand name Sumycin among others, is an antibiotic used to treat a number of infections. This includes acne, cholera, brucellosis, plague, malaria, and syphilis. It is taken by mouth.

Ciprofloxacin chemical compound

Ciprofloxacin is an antibiotic used to treat a number of bacterial infections. This includes bone and joint infections, intra abdominal infections, certain type of infectious diarrhea, respiratory tract infections, skin infections, typhoid fever, and urinary tract infections, among others. For some infections it is used in addition to other antibiotics. It can be taken by mouth, in eye drops, or intravenously.

Sepsis life-threatening organ dysfunction triggered by infection

Sepsis is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. Common signs and symptoms include fever, increased heart rate, increased breathing rate, and confusion. There may also be symptoms related to a specific infection, such as a cough with pneumonia, or painful urination with a kidney infection. In the very young, old, and people with a weakened immune system, there may be no symptoms of a specific infection and the body temperature may be low or normal, rather than high. Severe sepsis is sepsis causing poor organ function or insufficient blood flow. Insufficient blood flow may be evident by low blood pressure, high blood lactate, or low urine output. Septic shock is low blood pressure due to sepsis that does not improve after fluid replacement.

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. [3]

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]. [4] [5] 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. [6]

See also

Notes

  1. 1 2 Palmer & Reeder.
  2. DiBartolomeo. Page 14.
  3. DiBartolomeo (et alia).
  4. Hebra.
  5. Garrison & Morton (GM 3277).
  6. Frisch.

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