Rhinoscleroma

Rhinoscleroma : Chronic Granulomatous Disease of Nose

🌬 What is Rhinoscleroma?

Rhinoscleroma is a chronic, progressive granulomatous disease typically starting in the nose and spreading to other parts of the respiratory tract.

🦠 Causative Agent of Rhinoscleroma:

It is caused by a Gram-negative encapsulated bacillus called Klebsiella rhinoscleromatis.

What is another name of Klebsiella rhinoscleromatis?

🌍 Epidemiology of Rhinoscleroma:

• Named by Von Hebra, giving rise to the term "Hebra nose" for a characteristic appearance.

• Common in parts of Eastern Europe, Africa, and Latin America.

• In India, it is more common in the northern states of India compared to the southern states.

Which disease of nose is common in Southern states of India?

• Occurs at any age, but most common in the 2nd to 3rd decade of life.

• Slight female preponderance.

• Often associated with lower socioeconomic status, poor nutrition, and poor hygiene.

🔬 Pathogenesis of Rhinoscleroma:

• Exact mode of infection is unclear, but suspected to be airborne (inhalation of droplets or contaminated material).

• Patients are usually immunologically normal.

🦠 Pathology of Rhinoscleroma

Pathological findings can be described grossly and microscopically.

Gross Features:

• Involved tissues become indurated, resulting in a characteristic hard, "woody" feel on palpation (also known as a "woody nose").

What is the first site affected in Rhinoscleroma?

Why is Rhinoscleroma also called as Scleroma Respiratorium?

Microscopic Features (Histology):

Histological examination is diagnostic and reveals two key features:

1. Mikulicz Cells

2. Russell Bodies

Other microscopic features include dense infiltration of the submucosa by plasma cells, lymphocytes, and eosinophils.

What are Mikulicz cells and Russell Bodies?

🦠 Microbiology of Rhinoscleroma:

• Klebsiella rhinoscleromatis is a Gram-negative, encapsulated, non-motile diplobacillus.

• Microbiological diagnosis can be made by finding a positive culture on MacConkey Agar.

🚶 Clinical Features (Stages) of Rhinoscleroma

Rhinoscleroma is a progressive disease typically described in four stages:

1. Prodromal or Catarrhal Stage

2. Atrophic Stage

3. Granulomatous (Hypertrophic) Stage

4. Fibrotic (Cicatricial) Stage

What are the characteristic symptoms and signs of each stage of Rhinoscleroma?

🔬 Investigations of Rhinoscleroma

• Clinical Presentation: The classical appearance (especially in the granulomatous stage with woody feel and Hebra nose) is highly suggestive.

• Biopsy & Histopathology: The definitive diagnostic method. Biopsy of the nasal lesion confirms the diagnosis by identifying Mikulicz cells and Russell bodies.

• Diagnostic Nasal Endoscopy: To visualize the extent of the disease and involved structures.

• Imaging (X-ray, CT Scan)

What are the Radiological findings of Rhinoscleroma?

• Microbiological Culture: Culture of nasal discharge or biopsy material on MacConkey agar to isolate K. rhinoscleromatis.

🏥 Management of Rhinoscleroma

Management involves medical treatment to eradicate the bacteria and surgical intervention to address obstruction and deformities.

Medical Treatment:

• Antibiotics: The cornerstone of treatment.

  • Tetracycline (Drug of choice)
  • Streptomycin
  • Other options: Ciprofloxacin, Rifampicin.

What is the dose and duration of treatment of Tetracycline and Streptomycin?

What is the Criterion for stopping Antibiotics?

• Steroids

What is the role of Steroids in the treatment of Rhinoscleroma?

• Radiotherapy

What is the dose of Radiotherapy used in Rhinoscleroma?

What is the indication of Radiotherapy in Rhinoscleroma?

Surgical Treatment:

Aimed at removing granulation tissue, improving airway obstruction, and correcting deformities.

• Removal of granulomatous tissue with cautery or laser.

• Dilation of strictures in the fibrotic stage (e.g., subglottic stenosis). Placement of polythene tubes or spacers to maintain airway patency.

• Plastic surgery: Considered in the late cicatricial stage for cosmetic correction of deformities (Hebra nose, Tapir nose), only after confirming the disease is inactive (e.g., three consecutive negative biopsies).

💊 Complications of Rhinoscleroma

• External nose deformity
• Vestibular stenosis
• Strictures of soft palate, pharynx, larynx, trachea, bronchi
• Nasal regurgitation
• Anosmia
• Otitis Media with Effusion
• Malignant changes

What are some external nose deformities seen in Rhinoscleroma?

🤔 Differential Diagnosis of Rhinoscleroma

Consider the following conditions that can present with nasal/respiratory granulomas or similar features:

• Other Chronic Granulomatous Diseases involving the nose:

  • Tuberculosis
  • Leprosy
  • Lupus vulgaris
  • Syphilis (Gumma)
  • Rhinosporidiosis (friable, red, strawberry-like mass with sporangia)
  • Wegener's Granulomatosis (destructive)
  • Sarcoidosis (strawberry skin appearance)
  • Leishmaniasis
  • Eosinophilic Granuloma
  • Stewart's Granuloma (Midline Destructive Granuloma)

• Atrophic Rhinitis: Can resemble the atrophic stage of Rhinoscleroma (wide nasal cavities, crusts, atrophy), but lacks the characteristic induration, nodules, Mikulicz cells, and Russell bodies.

• Benign and Malignant Tumors presenting as nasal masses.