Categories
ORGAN SYSTEMS Otolaryngeal system (ENT)

Rhinoscleroma (RS)

Introduction

Chronic specific disease of nose and upper respiratory tract caused by Klebsiella rhinoscleromatis bacilli.

Aetiology

Klebsiella rhinoscleromatosis:

Short, encapsulated, and immotile Gram-negative bacillus with an affinity for nasal mucosa.
  • Transmission: Direct/indirect contact with nasal exudate of infected person
  • Disease predominantly occurs in middle-aged woman, living in poor hygienic and nutritional condition.
  • Involved sites:
    • Nasal cavity (M/C involved, 95–100%)
    • Nasopharynx (18–43%)
    • Larynx (15–40%)
    • Trachea (12%)
    • Bronchi (2–7%)

Clinical features

Catarrhal (rhinitic) stage:

Nonspecific rhinitis that evolves into chronic foul smelling purulent discharge, epistaxis, crusting, and nasal obstruction.

Granulomatous (proliferative/nodular) stage:

Clinical features are more prominent in granulomatous phase. In this phase, patient develops granulomatous nodular mass which leads to nasal obstruction. If left untreated patient goes into sclerotic phase.
  • Woody/Hebra nose

Sclerotic (cicatricial/fibrotic) stage:

Permanent complications are characteristic at this stage.
  • Nasal deformities
  • Anosmia
  • Oral anesthesia
  • Dysphonia, dysphasia, and stridor
(a and b) Hypertrophy of gums with verrucous lesion over lower lip | Malkud, S., & Mahajan, P. (2018). Rhinoscleroma: An Unusual Presentation. Indian dermatology online journal, 9(3), 191–193. https://doi.org/10.4103/idoj.IDOJ_134_17

Diagnosis

Tissue biopsy:

Histopathology is the mainstay in the diagnosis of rhinoscleroma. Intracytoplasmic bacilli can be demonstrated by special stains using Gram, PAS, Geimsa, Warthin-Starry, and Grocott’s silver methenamine stains. The detection of Type III Klebsiella antigen, CD 68 and altered CD4:CD8 ratio are helpful in the diagnosis.
  • Catarrhal stage: Nonspecific subepithelial infiltrate of polymorphonuclear leukocytes with granulation tissue
  • Granulomatous stage: Dense infiltration of lymphocytes, plasma cells, russell bodies, and pathognomonic large Mikulicz cells
    • Russell bodies: Homogenous eosinophilic inclusion bodies
    • Mikulicz cells: Atypical foamy monocytes containing causative bacilli
  • Sclerotic stage: Extensive fibrosis and less inflammatory cell infiltration
Dominance of plasma cells and squamous metaplasia (A), dominance of plasma cells and absence of esinophiles (B) and demonstration of Klebsiella Rhinoscleromatis bacilli within Mickulicz cells by H&E (B), PAS (C) and Geimsa (D) stains (arrows). Magnification is × 400 (A and D) and × 1000 (B and C). | Ahmed, A. R., El-Badawy, Z. H., Mohamed, I. R., & Abdelhameed, W. A. (2015). Rhinoscleroma: a detailed histopathological diagnostic insight. International journal of clinical and experimental pathology, 8(7), 8438–8445.

Management

Rhinoscleroma is a diagnostic and therapeutic challenge due to its chronic course, need for prolonged treatment and repeated relapses.

Antibiotic therapy:

Mainstay of treatment. Patient is kept under regular observation to monitor any incidence of recurrence.
  • Tetracycline (2g/day) + streptomycin (1g/day) for 1 month and continued till 2 consecutive negative biopsies
(a and b) Improvement of lesions after 4 months of treatment | Malkud, S., & Mahajan, P. (2018). Rhinoscleroma: An Unusual Presentation. Indian dermatology online journal, 9(3), 191–193. https://doi.org/10.4103/idoj.IDOJ_134_17

Surgical management:

Indicated in permanent deformities

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