Definition: Rhinoscleroma is a chronic granulomatous condition of the nose that can extend to the upper respiratory tract. It affects the nose mainly (90-95%) but it may also affect the nasopharynx, larynx, trachea, and bronchi.
Causative organism: Klebsiella Rhinoscleromatis (gram negative bacilli).
Epidemiology:
- More common in underdeveloped countries.
- Endemic in Egypt.
- Females more than males.
- More common between 10-30 years.
Pathology:
A) Atrophic stage: Similar to atrophic rhinitis, with chronic inflammatory cells.
B) Nodular stage: Submucosa shows increased vascularity & cellular infiltration:
- Lymphocytes, macrophages, plasma cells.
- Mikulicz cells: large vacuolated cells with foamy cytoplasm and small central nucleus, bacilli present within the vacuoles.
- Russel bodies: Plasma cells with hyaline degeneration, bright red with no nucleus.
C) Fibrotic stage: Dense fibrosis, reduced vascularity & cellularity
Clinical picture of Scleroma
Insidious onset, slow course, several stages, may be more than one stage at a time (1&2 are active, while 3 is inactive).
A) Atrophic stage:
- Similar to atrophic rhinitis.
B) Nodular stage:
- Symptoms: obstruction – discharge – epistaxis
- Signs: Bilateral submucosal mucocutaneous nodules.
- First red and soft, then pale and hard
- May coalesce together to form large masses.
C) Fibrotic stage:
- Symptoms: nasal obstruction.
- Signs: Dense fibrosis, narrowing of nasal cavities, hard alae.
- It may affect nose or extend to the nasopharynx, hard palate, subglottic and trachea.
Diagnosis of Scleroma:
Depends mainly on clinical examination and biopsy to reveal the characteristic pathological feature, extremely difficult in atrophic stage. Electron microscopy usually needed.
Sequalae:
- Extension: nasal tip, columella, upper lip, lacrimal sac (dacryoscleroma), Pharynx, larynx and nasal dorsum.
- Fibrosis → deformity, 2ry atrophic rhinitis.
Treatment of Scleroma:
Treatment should also include long-term antimicrobial therapy and surgical intervention in patients with symptoms of obstruction.
Scleroma responds to treatment with ciprofloxacin, Gemifloxacin, rifampicin (600 mg daily, it is hepatotoxic and leads to red urine), and streptomycin (1gm daily IM, it is oto and nephrotoxic). Long-term antibiotic therapy often is needed to eradicate this infection.
The choice of long-term antibiotic therapy should be guided by the patient's age and sex.
Treatment is stopped only when two consecutive cultures from the biopsy material are negative.
Surgical treatment may be required to establish the airway and correct nasal deformity.