Rhinosleroma

RHINOSCLEROMA

• Rhinoscleroma is a progressive granulomatous disease of the upper respiratory tract.
• It is caused by the gram-negative coccobacillus K rhinoscleromatis or Frish Bacillus.
• The disease probably begins in areas of epithelial transition such as the vestibule of the nose, the subglottic area of the larynx, or the area between the nasopharynx and oropharynx. 
• It is a chronic infection of the nose characterised by sclerosis and stenosis of the nasal passage.
• Rhinoscleroma may be found in all age groups, but typically young adults 20–30 years old are most frequently affected.
•  Both sexes are equally affected.
• The disease is endemic in several parts of world.
• In India, it is seen more often in northern than in the southern parts.
•  Airborne transmission combined with poor hygiene, crowded living conditions, and poor nutrition contributes to its spread.

PATHOLOGY OF RHINOSLEROMA

• The disease is characterized by presence of granulations which infiltrate the submucosa ,with scattered Mikulicz cells(large vacuolated foamy cells containing a central nucleus and acid fast Frisch bacilli),Rusell bodies(resmbling plasma cells with an eccentric nucleus and deep eosin staining cytoplasm) and lymphocytes in dense fibrous tissue.

CLINICAL FEATURES IN A CASE OF RHINOSCLEROMA

SYMPTOMS

• Blocking of the nose,Nasal Discharge,Headache,Epistaxis

SIGNS

• External nose is firm and woody to touch.
• Anterior Rhinoscopy:Throat:may show extension of the sclerosing process downwards from the nose to the oropharynx,larynx.

3 stages:

1. Atrophic Stage:is present initially,with crusting ,roomy cavity and atrophic dry nasal mucosa.
2. Nodular stage:Bluish red non-ulcerative polypoidal masses of rubbery consistency are formed.
3. Fibrotic stage of stenosis:of the nasal passages varying from partial obstruction to total atresia . 

TREATMENT IN A CASE OF RHINOSCLEROMA

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

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

  Exam Question
   
  • Mikulicz cells and Russell bodies are seen in Rhinoscleroma.
  • A roomy nasal cavity and atrophic dry nasal mucosa with thick crust formation and woody hard external nose is seen in Rhinoscleroma.
  • Rhinoscleroma is caused by Klebsiella Rhinoscleromatis.
  • Rhinoscleroma is a slowly progressing granulomatous disease.
  • Rhinoscleroma is most common in young adults 20–30 years old.
  • Poor nutrition contributes to the spread of Rhinosleroma.
  • Drug of choice in Rhinoscleromatosis is Tetracycline.
  • Frish bacillus causes Rhinoscleroma.