*Corresponding author:
Daniel Xia
Division of Hematopathology and Transfusion Medicine
University Health Network
200 Elizabeth Street
Toronto, Ontario M5G 2C4
Email: Daniel.xia@uhn.ca
Phone: 416-340-4800 x4734
Consent information: Written informed consent was obtained from
the patient to publish this report in accordance with the journal’s
patient consent policy.
Abstract: Rhinoscleroma is a granulomatous infectious disease
that typically affects the nasopharynx and upper respiratory tract.
Non-endemic (e.g., North American) and laryngeal cases are rare. This
case highlights the importance of pathognomonic Mikulicz cells for
diagnosis.
A man in his early-50s presented with worsening stridor,
shortness-of-breath, and hoarseness over 2 years. Originally from
northwestern South America, he reported with similar symptoms 7 years
ago, but work-up did not produce a specific diagnosis. For this
presentation, a large obstructing mass on the laryngeal surface of the
epiglottis was identified on emergency laryngoscopy that prevented
visualization of the vocal cords and lower respiratory tract (Figure 1).
Tracheostomy was performed. Biopsies showed squamous mucosa with
submucosal fibrosis and a dense infiltrate, composed mostly of plasma
cells and plasmacytoid lymphocytes, with rare/scattered macrophages
containing intracellular gram-negative rods (Mikulicz cells; Figure 1)
–suggesting a diagnosis of rhinoscleroma1,2.
Confirmatory cultures showed Klebsiella ozaenae . Subsequent
ciprofloxacin led to improved stridor, near-complete regression of the
supraglottic mass, with improved vocal cord mobility and patent
subglottis; corking trials were carried out with the tracheostomy
tube, and the patient was safely decannulated. At 8-month follow-up,
the laryngeal exam remains stable with no evidence of
recurrence/regrowth.