RHINOSCLEROMA NOTES

 

INTRODUCTION

Chronic granulomatous infectious disease primarily affects upper respiratory tract.

 

EPIDEMIOLOGY

 

Affects all ages-young, first 3 decades, world wide distribution ,  low socioeconomic background with poor living conditions , malnutrition.

HISTORICAL IMPORTANCE

• For the first time VON FRISH identified the causative organism of the disease RHINOSCLEROMA.
• MIKULICZ has described the classical FOAMY cells in the histopathology of            RHINOSCLEROMA. 
• VON HERBA discovered the disease , after his name HERBA NOSE.
• VON MIKULICZ studied the histopathology of disease.

CAUSATIVE AGENT

KLEBSIELLA RHINOSCLEROMATIS ( Frisch bacillus ) – 2.5 um , capsulated ,gram negative , immotile organism belongs to ENTEROBACTERIACEAE FAMILY.

ROUTE OF TRANSMISION

Through droplet spread.

 

LOCALIZATION

Nasal cavity

Palpebral conjunctiva

Middle ear

Nasopharynx

Tracheobronchial tree

Esophagus

Larynx

Pharynx

DIAGNOSIS

Biopsy of the involved site , demonstration of bacilli with special stains ,supplemented by immunohistochemistry.

 

IMAGING

• Intracranial extension of the disease due to destruction of posterior wall of left sphenoidal         sinus by CT SCAN.
• MRI detects nasal mass extending into nasopharynx.

GROSS PATHOLOGY

Infected mucosa is pale in appearance & demonstrates diffuse nodular thickening(FLORID PHASE).

 

MICROSCOPY

1.Squamous metaplasia with inflamed granulation  tissue is seen in early stages of the disease.

 

2.FLORID/PROLIFERATIVE PHASE:

submucosal granulomatous infiltrate composed of macrophages with clear foamy cytoplasm (MIKULICZ CELLS) intimately associated with admixture of lymphocytes & numerous plasma cells.rod shaped gram negative organisms can be demonstrated in MIKULICZ cells.

3.FIBROTIC/RESOLUTION PHASE: 

no Mikulicz cells , extensively replaced by fibrosis and lymphocytes and plasma cells (russel bodies – accumulation of immunoglobulins).

HIGH POWER IMAGE SHOWA MIKULICZ CELLS WITH ABUNDANT FOAMY CYTOPLASM.

 

SPECIAL STAINS

GIEMSA , WARTHIN STARRY STAIN, Dieterle stain    & PAS positive (inclusion bodies ).

IMMUNOHISTOCHEMISTRY

MIKULICZ CELLS-CD68 positive.

Langerin,S100,CD1a negative.

ELECTRON MICROSCOPY

Bacilli and granular material is seen in large phagosomes.

 

DIFFERENTIAL DIAGNOSIS

Syphilis- plasma cells ,detection of Treponema pallidum by dark field microscopy.

Rosai dorfmann disease - sinus histiocytosis with massive lymphadenopathy , emperiploesis.

Inflammatory pseudotumour/plasma cell granuloma-along with mixed inflammatory infiltrate , plasma cells , vascular proliferation there is storiform pattern of arrangement of fibroblasts/myofibroblasts.

Wegners granulomatosis( triad of focal glomerulonephritis , vasculitis and necrosis of Upper respiratory tract) now named as GRANULOMATOSIS WITH POLYANGIITIS.

Malignant lymphoma - clonal disorder , absence of mikulicz cells , Immunohistochemistry helps in doubtful cases.

MANAGEMENT

Long term antibiotics followed by surgical debridement

Surgical resection

Co2 laser surgery

OUTCOME

Slowly progressive disease with relapses and recurrences.

Fatal if obstructs trachea.