Rhinoscleroma

RHINOSCLEROMA
Causativ organism: 
  • Chronic granulomatous disease caused by Gram negative bacillus called Klebsiella rhinoscleromatis (Frisch bacillus) Stages of disease progression:
  • 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.
  • The disease runs through the following stages:v
  1. Catarrhal or atrophic stage: It resembles atrophic rhinitis and is characterized by foul smelling purulent nasal discharge and crusting.
  2. Granulomatous stage: Granulomatous nodules in nasal mucosa. There is also subdermal infiltration of lower part of external nose and upper lip giving a 'woody' feel. Nodules are painless and non-ulcerative.
  3. Cicatricial stage: This causes stenosis of pares, distortion of upper lip, adhesions in the nose, nasopharynx and oropharynx. There may be subglottic stenosis with respiratory distress
Clinical and Histologic features:
  • Rhinoscleroma may be found in all age groups, but typically young adults 20–30 years old are most frequently affected. 
  • Airborne transmission combined with poor hygiene, crowded living conditions, and poor nutrition contributes to its spread.
  • Subdermal infiltration of the lower part of external nose and upper lip giving a woody feel
  • Miculicz' cells (macrophages containing large amounts of bacteria-filled vacuoles) and Russell bodies (spherical structures found in the cytoplasm of plasma cells and also seen in multiple myeloma)
  • Corin cells, morula cells, mott cells are also seen
  • Gothic sign
  • Potato nose, hebra nose, elephantiasis of nose
  • Tapir nose (also in leishmaniasis)
Treatment: streptomycin + tetracycline for 4-6 weeks

Exam Question
Cause:
  • Chronic granulomatous disease caused by Gram negative bacillus called Klebsiella rhinoscleromatis (Frisch bacillus)
  • Clinical and Histologic features:
  • Rhinoscleroma may be found in all age groups, but typically young adults 20–30 years old are most frequently affected. 
  • Airborne transmission combined with poor hygiene, crowded living conditions, and poor nutrition contributes to its spread.
  • Subdermal infiltration of the lower part of external nose and upper lip giving a woody feel
  • Miculicz' cells (macrophages containing large amounts of bacteria-filled vacuoles) and Russell bodies (spherical structures found in the cytoplasm of plasma cells and also seen in multiple myeloma)
Treatment: streptomycin + tetracycline for 4-6 weeks

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