Table of Contents
Definition / general | Treatment | Case reports | Microscopic (histologic) description | Positive stains | Electron microscopy description | Differential diagnosisCite this page: Pernick N. Rhinosclerosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/larynxrhinosclerosis.html. Accessed December 27th, 2024.
Definition / general
- Rare; chronic granulomatous disease of nasal cavity (95 - 100%), nasopharynx (18 - 43%), larynx (15 - 40%), trachea (12%) or bronchi (2 - 7%) caused by Klebsiella rhinoscleromatis
- Usually low socioeconomic environments of central / South America, Africa, Middle East, Philippines and India; rare in US (usually immigrants)
- Most common in young adults
- Slowly progressive with remission and relapse; not fatal unless it obstructs the airway
- Microbiology: MacConkey agar cultures are 50 - 60% sensitive; bacteria is gram negative, encapsulated, nonmotile, diplobacillus, member of Enterobacteriaceae, not normal flora, infective via drops or contamination of material that is inhaled
Treatment
- Antibiotics for months to years
- Possibly steroids, surgery to treat airway compromise and tissue deformity
Case reports
- 32 year old woman with supraglottic granulomas (Arch Pathol Lab Med 2001;125:159)
Microscopic (histologic) description
- Catarrhal / atrophic, granulomatous and sclerotic stages
- Initially: squamous metaplasia and inflamed granulation tissue
- Later: pseudoepitheliomatous squamous hyperplasia with foamy macrophages (Mikulicz cells containing bacteria), plasma cells with Russell bodies and granulomatous inflammation
- Late: fibrosis, lymphocytes and plasma cells but no Mikulicz cells
Electron microscopy description
- Large phagosomes containing bacilli and finely granular material (antibodies on bacterial surface and aggregates of bacterial mucopolysaccharides)
Differential diagnosis
- Leprosy
- Other granulomatous processes
- Rosai-Dorfman disease