Tuesday, July 4, 2017

Mikulciz Disease- Rhinoscleroma

Mikulciz disease aka Rhinoscleroma is a chronic granulomatous disease commencing in the nose and extending into the nasopharynx, oropharynx, larynx, trachea and bronchi

This disease may occur in either or the sexes at any age.

Causative organism- Klebsiella rhinoscleromatis (gram negative Frisch bacillius)

Three stages-

1. Atrophic Stage-
This stage typically resembles atrophic rhinitis and presents with nasal discharge which is purulent and foul smelling with nasal crusting.

2. Granulomatous Stage-
This is a proliferative stage. Characterised by granulomatous reaction and presence of mikulciz cells.
Presents as painless nodules in the nasal mucosa.
The subdermal infiltration into the lower part of external nose and upper lip gives a Woody feel to the nose.
There may occur broadening of the nose with thickened skin- Hebra nose

3. Cicatricial Stage-
There occurs fibrotic adhesions and fibrosis of nose, nasopharynx and oropharynx.
The fibrotic deformity of nose is known as- Taper nose.

Most common symptoms-
Nasal discharge and crusting> nasal deformity> epistaxis

Diagnosis-
Diagnosis can be done histopathologically which shows mikulciz cells and Russell bodies in the submucosa which is characteristic of rhinoscleroma.
Mikulciz cells are foam cells with central nucleus and vacuolated cytoplasm containing bacteria.
Russell bodies are eosinophilic inclusion bodies seen in the cytoplasm.

Treatment-
Streptomycin (2g/day) + teracycline (2g/day) for 4-6 weeks

Hope this helps!
Ashita Kohli

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