Larynx and hypopharynx
Inflammatory / infectious lesions
Rhinosclerosis

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

Revised: 19 March 2018, last major update November 2013

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Rhinosclerosis larynx

Cite this page: Pernick, N. Rhinosclerosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/larynxrhinosclerosis.html. Accessed July 16th, 2018.
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
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
Microscopic (histologic) images

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Fig 1: underlying submucosa with diffuse granulomatous inflammation
Fig 2: multiple, large, vacuolated macrophages filled with bacilli
Fig 3: gram negative bacteria

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