Rhinosleroma

• Rhinoscleroma, or simply scleroma, is a chronic granulomatous bacterial disease of the nose that can sometimes infect the upper respiratory tract.
•  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.

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).

• Rhinoscleroma is caused by the gram-negative coccobacillus K rhinoscleromatis.

The disease begins in areas of epithelial transition such as the vestibule of the nose, the subglottic area of the larynx. It resembles atrophic rhinitis in atrophic stage. Subdermal infiltration of lower part of external nose and upper lip gives a ‘woody’ feel. 

The classic biopsy findings compatible with rhinoscleroma include Mikulicz cells, macrophages containing large amounts of bacteria-filled vacuoles, and Russell bodies, spherical structures found in the cytoplasm of plasma cells. 

Mikulicz cells are large mononuclear histiocytes with vacuolated cytoplasm containing causative bacilli and Russell bodies are homogenous eosinophilic inclusion bodies found in the plasma cells.

Rhinoscleroma:
It is a rare, chronic progressive granulomatous disease of the upper respiratory tract caused by Klebsiella rhinoscleromatis.

In rhinoscleromatosis, organism may be difficult to eradicate, despite aggressive therapy.

A combination of conservative surgical debridement and long-term antibiotic coverage is the mainstay of therapy for rhinoscleroma.

Tetracycline has been shown to be effective and inexpensive for patients unless contraindicated.

Fluoroquinolones may be used as an alternative, given their excellent gram-negative activity and convenient dosing regimen

Rhinoscleroma is a rare chronic (slowly progressive) inflammatory condition caused by bacteria called Klebsiella rhinoscleromatis.

Ans. A. Rhinoscleroma

RHINOSCLEROMA:

• It is a chronic granulomatous disease caused by Gramnegative bacillus called Klebsiella rhinoscleromatis or Frisch bacillus.

CLINICAL FEATURES:-

The disease runs through the following stages: 

1. Atrophic stage: It resembles atrophic rhinitis and is characterized by foul-smelling purulent nasal discharge and crusting.

2. Granulomatous stage: Granulomatous nodules form in the nasal mucosa. There is also subdermal infiltration of the lower part of the external nose and upper lip giving a “woody” feel. Nodules are painless and nonulcerative.

3. Cicatricial stage: This causes stenosis of nares, distortion of the upper lip, adhesions in the nose, nasopharynx and oropharynx. There may be subglottic stenosis with respiratory distress.

Rhinoscleroma is a chronic granulomatous disease caused by Gram negative bacillus called Klebsiella rhinoscleromates or Frisch bacillus

Ans. is ‘c’ i.e., Klebsiellarhinoscleromatis infection

Rhinoscleroma

• The causative organism is Klebsiellarhinosclerontatisor Frisch bacillus, which can be cultured from the biopsy material.
• The disease is endemic in several parts of world.
• In India, it is seen more often in northern than in the southern parts.
• Biopsy shows infiltration of submucosa with plasma cells, lymphocytes, eosinophils, Mikulicz cells & Russell bodies.
• The latter two are diagnostic features of the disease.
• The disease starts in the nose & extends to nasopharynx, oropharynx, larynx, trachea & bronchi.
• Mode of infection is unknown.
• Both sexes of any age may be affected.