Scleroma

Prof.D M El-Hennawi
 Scleroma
Definition: It is a chronic progressive
granulomatous inflammatory disease of the
respiratory track, described by Von Hebra.

This disease is endemic in Egypt and may other

countries. It was called’’ Rhinoscleroma”.
 Scleroma
Aetiology
Klebsiella rhinoscleromatus proved by

1- regular incidence in the affected tissues

2- experimental inoculation in nose and

respiratory tract
 Scleroma
Incidence
Age : 2nd,3rd decades prolonged exposure

Family incidence: close contact, long period

Sex incidence: female>male

 Scleroma
Sites of the disease
It starts in the nasal cavity then it extends
backward.

It arises at the MCJ in the nose and larynx

There are RARE abnormal sites; orbit, middle

ear, skin, IC.
 Scleroma
Pathology
Metaplasia -- followed by -- Dense cellular
infiltrate
Mickulicz cells :A granules antibodies ,B
granules antigen
Russel bodies: eosinophilic homogenous bodies
Mott cells: plasma cells with inclusion bodies
 Scleroma
Pathology
• Plasma cells play a double role in pathogenesis
of scleroma
1st normal function
2nd transform to Mickulickz cells---shelter for
K.rhinoscleromatis(intracellular)
The progress of the disease depends on the
balance between these two processes
 Scleroma
Pathology
The final cure of scleroma is achived if the
medication could penetrate the defensive
barrier of Mickulicz cells
 Clinical picture and Stages
• Catarrhal stage :
1) prolonged purulent rhinorrhoea
2) dense cellular infiltration of sub mucosa

• Atrophic stage
metaplasia of nasal mucosa so it appears thin
dry covered with crust either 1ry or follow R/
 Clinical picture and stages
• Granulomatus stage
1) Small soft granulomatous nodules ---enlarge
coalease to form firm nodules
2)During this stage organism frequently
isolated and pathology is most characteristic
• Sclerotic stage
dense fibrotic narrowing the nose ,larynx
,pharynx
 Clinical picture
• Uvula sign—diagnostic but absence is not
Grade 1:dimple at base of uvula in the act of
gag---superficial infiltration
Grade 2:dimple present at rest, on contraction
uvula points forward
Grade 3:exagerated grade 2 but long axis of uvula
is directed forward
Grade 4: absent uvula
 Clinical Picture
• Laryngeal Scleroma: 2ry to nasal
1) Granulomtus bands appear anteriorly and
fades posterioly below and parallel to vocal
folds, either attached or separated from folds
2)Granulomas might lead to narrowing of
subglottic region. Lately it will be due to
fibrosis and crust formation
3)Epiglottis:deviated,atrofic
4)Arytenoid process unequal size
 Clinical Picture
• The patient will complain if:
Hoarseness of voice
Cough
Stridor
Crust expectoration &mild haemoptysis
 Clinical Picture
• Tracheal &Bronchial scleroma
Usually 2ry to nasal scleroma

laryngeal scleroma either diffuse or localized

in the form of granulomtous or atrophic types.

 Diagnosis of scleroma
• History long history of nasal troubles in a
patient from endemic area
• Clinical picture Multi system affection
• Biopsy Mickuliz cells ,Roussels bodies and
intracellular organism
• Bacteriological study: tissue grinding not nasal
swab
 Diagnosis
• Serological testing

• Radiological study

• Endoscopy

• Differential Diagnosis
Atrophic stage :Ozena, syphilis,lupus vulgaris
Granulomatus stage:syphilis,lupus vulgaris,tumour
 Treatment of Scleroma
• Conservative treatment
a)Antibiotic according to culture and sesitivity
(tissue grinding) both systemic and local:e.g
Rifampicin,6oomg/day/6 weeks and more
effective if compined with tetracyclines.
b)Dilation of larynx ,tracheobronchial
tree,endoscopic or with CO2 laser
 Treatment of Scleroma
• Surgical treatment
a)Tracheastomy
b)Excision of granulomatous mass in the nose
and PNS,this gives symptomatic relief and
improve the medical therapeutic
results:endoscopic or CO2 laser
c)Dilate stenosed nose in the fibrotic stage
d)Pulmonary lobectomy