Nasal cavity, paranasal sinuses, nasopharynx
Inflammatory / infectious lesions
Rhinoscleroma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 18 May 2018, last major update November 2004

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Rhinoscleroma[TI] nasal

Cite this page: Pernick, N. Rhinoscleroma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalrhinoscleroma.html. Accessed August 19th, 2018.
Definition / general
  • Also called scleroma
  • Rare; chronic granulomatous disease affecting 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, India; rare in U.S. (usually immigrants)
  • Most common in young adults
  • Initially nonspecific rhinitis, then purulent, fetid rhinorrhea with crusting; then granulomatous stage with blue-red granular nasal mucosa with intranasal rubbery nodules or polyps, epistaxis, anosmia, enlargement of uvula, anesthesia of soft palate, variable airway obstruction; finally sclerosis
Treatment
  • Tetracycline or trim sulfa, possibly steroids, surgery to correct scars or stenosis
Microbiology
  • Bacteria is gram negative, encapsulated, nonmotile diplobacillus, member of Enterobacteriaceae, not normal flora, infective via drops or contamination of material that is inhaled
  • MacConkey agar cultures are 50 - 60% sensitive
Case reports
Microscopic (histologic) description
  • Initially inflamed granulation tissue
  • Later hyperplastic mucosa with pseudoepitheliomatous squamous hyperplasia, granulomatous inflammation, foamy macrophages (Mikulicz cells containing bacteria) and plasma cells with Russell bodies
  • Variable vasculitis and ulceration
  • Late fibrosis, lymphocytes and plasma cells but no Mikulicz cells
Microscopic (histologic) images

Images hosted on other servers:

1. Diffuse granulomatous inflammation
2. Vacuolated macrophages with bacilli (Mikulicz cells)
3. Gram negative bacteria by gram stain

Positive stains
  • PAS, Giemsa, Steiner or Hotchkiss-McManus stains for gram negative bacteria
Electron microscopy description
  • Large phagosomes containing bacilli and finely granular material (antibodies on bacterial surface and aggregates of bacterial mucopolysaccharides)
Differential diagnosis