What is Rhinoscleroma or Hebra NOSE

Rhinoscleroma is also known as WOODY NOSE or Hebra Nose or Balkan leprosy

1. First described by Hebrew.
2. Termed by Von hebra.
3. Chronic granulomatous  disease  of nose.

Causative agents of Hebra Nose

Kliebseilla Rhinoscleromatis(Firsch bacilli –gram negative diplococci).

Sites of infection

1. Nasal cavity:- Inferior turbinate> middle turbinate> anterior wall of nasal septum.
2. Nasopharynx.
3. Oropharynx.
4. Larynx:- subglottic> glottis.
5. Trachea(cervical).
6. Bronchus

Stages of Disease

1. Catarrhal stage:- mucopurulent, foul  smelling discharge,occasionally blood tinged – CARPENTER’ S GLUE.
2. Atrophic  stage:- mucosal atrophy, crustations, pinkish mucosa, Honeycomb color crusting. Diagnosed by Immune Compliment fixation test- Levin test.
3. Grannulomatous stage/ nodular:- painless ,non tender,small granulomatous nodules,non ulcerating nodules:-Russel bodies, Mikulicz cells.
4. Cicatarisation/ sclerotic/fibrosis/stenosis stage:- Typical histological picture is granulomatous changes in submucosa- TAPIR Nose.

RHINOSCLEROMA Histopathology

1-Diffuse granulomatous inflammation

2-Vacuolated macrophages with bacilli (Mikulicz cells)

3-Gram negative bacteria by gram stain (in the follwing figure)

Rhinoscleroma OSCE Slides

 Signs and symptoms

Nasal discharge, epistaxis, nasal obstruction, anosmia,hoarseness of voice,middle ear problems, narrow nasal cavity.

Asthetic deformity:- Alar cartilages,tip of the nose.

Complications

1. Tracheal stenosis.
2. Cicatrization of oropharyngeal  structure.

Pathological Findings

1. Mikulicz cells– large foamy vacuolated histocytes.
2. MOTT /UNN BODY/MORULA CELL/CORIN CELL– Active  accumulation of RUSSEL BODIES.( plasma cells)

INVESTIGATIONS:-

1. CBC
2. Cultures:- not diagnostic .sample taken from nodules/ secreations.
3. Biopsy and Histopathological- Definative diagnostic.
4. X –ray:- nose,pns,necksofttissue,chest(bronchus,trachea),Ear(eustachain tube).

Gothic sign

Fibrosis of pharyngeal walls resembles church.

V sign

on palatal region.

• Indirect laryngoscopy:- atrophy of larynx, subglottic stenosis.

TREATMENT

• Medical management:-Aminoglycosides(streoptomycin),Tetracyclines,Cephalosporines,Septran,Rifampicin(1%),Arciflavin(2%),Chlormycetin
• Kaliasha Regime:-  Carbolic acid+ glacial acetic  acid + glycerine.
• Lasers.
• Radiations:- 3000-3500 cGy.
• Cryosurgery.
• Electrocautery.
• Dilation and Tracheotomy.
• Plastic reconstruction :-  flaps, rhinoplasty considered only in stage 2,3 of disease once patient declared free of active disease by  atleast 3 consecutive biopsies  negative for disease.

Read Other Nose DNB ENT Questions

You can remember these cells by Russell Peters

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