May 24, 2023

EDUCATIONAL PEARL

Rhinoscleroma: Chronic infectious granulomatous disease of the paranasal sinus

  • Presenting Symptoms: Nasal obstruction (polyps), rhinorrhea, anosmia, epistaxis, nasal deformity; may involve other portions of airway

  • Epidemiology: Endemic to eastern Europe, East Africa, North Africa, Indian subcontinent, Latin America. Some suggest a genetic predisposition

  • Etiology: Unclear; suggested risk factors include: EBV, tobacco use, autoimmune reaction, radiation

  • Pathophysiology:

    1. Catarrhal: Persistent, purulent, foul-smelling rhinorrhea, nasal honey-colored crusting that last weeks

    2. Granulomatous: Non-tender granulomatous nasal masses (may also included lower airway such as glottis, subglottis); obstruction, epistaxis, destruction of septum, thickened soft palate

    3. Fibrotic: Lesions heal with extensive scar tissue formation (fibrotic narrowing of nasal passage)

  • Histopathology: Mikulicz cell (foamy-histiocytes with intracellular inclusion bodies), Russell bodies, granulomas, and pseudoepitheliomatous hyperplasia

  • Diagnosis: Biopsy, culture, serum antibodies

  • Treatment: Long-term antibiotics determined by culture sensitivities (often tetracycline or fluoroquinolone), debridement, laser excision, bronchoscopy

Rhinologic exam

Moraes MA, Magalhães AV, Marinho LC, Azevedo AE, Carneiro FP, Raymundo IT. Rhinoscleroma causing severe bilateral nasal obstruction. Braz J Infect Dis. 2010 Mar-Apr;14(2):190-2.

HE staining micrograph showing large vacuolated histiocytes (Mikulicz cells) and plasma cells with Russell bodies 

Mukara BK, Munyarugamba P, Dazert S, Löhler J. Rhinoscleroma: a case series report and review of the literature. Eur Arch Otorhinolaryngol. 2014 Jul;271(7):1851-6.

Coronal CT (note heterogeneity)
Ibrahim D, Rhinoscleroma. Case study, Radiopaedia.org

Further Readings:

 General 

General 2

Disease Chronicity

Written by: Graham Pingree