Rhinoscleroma: chronic granulomatous disease of the nose #otorhinolaryngology #ENT #rhinoscleroma

1. RHINOSCLEROMA
DR BHAGYASHREE DESHMUKH
NANAVATI MAX SUPERSPECIALITY HOSPITAL, MUMBAI

2. INTRODUCTION
 First described: Von Hebra 1870
 Histologic features: Johan Von Mickulich 1877
 Identified organism: Von Frisch 1882
 Chronic granulomatous disease
 Affecting especially nose and upper respiratory tract
 All races
 Both genders (more in females)
 Age: middle aged

3. ETIOLOGY
 Klebsiella rhinoscleromatis: short, encapsulated, immotile, intra-
cyctoplasmic coccobacillus (Frisch bacillus)
 Humans are the only hosts
 Contraction: direct inhalation of droplets or contaminated material
 Facilitated by poor hygiene, malnutrition
 Occurs at epithelial transition zones

4. PATHOPHYSIOLOGY
 Altered immune response
 Cellular immunity impaired; humoral immunity intact
 Mucopolysaccarides in bacterial capsule inhibit its phagocytosis
 Cause of chronicity: altered CD4/CD8 ratio

5. CLINICAL FEATURES: Based on 3 stages
 I. Catarrhal: Rhinitic stage
- Nasal obstruction (m/c)
- Crusting
- Discharge (carpenter’s glue)
- Epistaxis

6.  II. Granulomatous/ Proliferative/ Nodular:
 More prominent clinical features
 Painless nodular masses
 Nodules have cartilage like consistency
 m/c- seen adhering to septum with relative sparing of sinuses
 Middle and inferior turbinates are m/c affected
 Other sites: nasopharynx, oral cavity, paranasal sinuses, larynx, lips,
trachea, bronchi.

7. NASAL MASSES WITH WOODY NOSE AND UPPER LIP
Other: Tapir Nose

8.  III. Sclerotic/ Cicatrical/ Fibrotic Stage:
 If untreated, progresses to extensive cicatrization
 Scarring, adhesions, puckering of nose, soft/ hard palate leads to
distortion of face
 Anosmia
 Dysphonia
 Stridor

9. DIAGNOSIS:
 Tissue culture: Mac conkey Agar
 Stains: Gram, PAS, Giemsa, Warthin, Starry Grocott’s
methanamine: Intracytoplasmic bacilli
 Type III Klebsiella antigen with CD68 : IHC
 Imaging: similar to fungal or neoplastic findings (soft tissue
masses, expansion of cavity and bony erosions)
 Good for surgical planning

10. DEFINITE: HISTOPATHOLGY
 Catarrhal- subepithelial infiltrate of PMN and granulation tissue
 Granulomatous: most conspicuous
- Dense infiltration of lymphocytes, plasma, cells, Russell bodies,
Mickulicz cells
- MICKULICZ Cells- macrophages with clear cytoplasm, contain
bacilli
- RUSSELL Bodies- bloated plasma cells with inclusions
 Sclerotic stage: less inflammatory cell infiltration

12. TREATMENT
 Medical:
- Third generation Cephalosporins and Doxycycline long term use
- Sclerotic lesions: respond well to Ciprofloxacin
- IM Streptomycin 1g (4-6weeks)
- Treatment can be stopped after 2 consecutive biopsies are negative
 Surgical:
- Extensive granulomatous lesions require surgery
- Plastic surgery is considered in cicatrical stenosis or when imperforations persist
in nose and PNS/ pharynx/ larynx.