Rhinoscleroma

📌 Definition: A chronic granulomatous disease caused by Gram-negative bacillus Klebsiella rhinoscleromatis (also called Frisch Bacillus).

Epidemiology: Endemic in parts of the world. In India, common in Northern parts.

Pathology

• Site: Starts in Nose → extends to Nasopharynx → Oropharynx → Larynx (Subglottic) → Trachea.
• Host: Both sexes, any age. Mode of infection is unknown.

Clinical Stages

The disease typically runs through three stages:

1. Atrophic Stage

• Resembles Atrophic Rhinitis.
• Features: Foul-smelling purulent nasal discharge and crusting.

2. Granulomatous Stage

• Nodules form in nasal mucosa (painless, non-ulcerative).
• "Woody" Feel: Subdermal infiltration of lower nose and upper lip.

3. Cicatricial Stage

• Fibrosis & Stenosis: Causes stenosis of nares.
• Adhesions: Nose, nasopharynx, and oropharynx.
• Danger: May cause Subglottic Stenosis → Respiratory distress.

Diagnosis (Biopsy)

Biopsy shows plasma cells, lymphocytes, and eosinophils. Two specific features are diagnostic (Table):

Diagnostic Feature	Description
1. Mikulicz Cells	Large Foam Cells with a central nucleus and vacuolated cytoplasm containing the causative bacilli.
2. Russell Bodies	Homogenous eosinophilic inclusion bodies found inside plasma cells. (Accumulation of immunoglobulins).

Treatment

Medical Therapy

• Regimen: Streptomycin (1g/day) + Tetracycline (2g/day).
• Duration: Minimum 4–6 weeks (repeat if necessary).
• Endpoint: Treatment stopped only when 2 consecutive biopsy cultures are negative.
• Adjunct: Steroids (to reduce fibrosis).

Surgical Therapy

• Required to establish airway (if stenosed) and correct nasal deformity.