Unidade de Infecciologia, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE
Abstract
Introduction: Rhinoscleroma is a rare, chronic, granulomatous disease that most
frequently affects the upper respiratory tract, especially the nasal cavity and sometimes extends through the lower respiratory tract. Is associated with Klebsiella rhinoscleromatis, which is endemic in certain geographic regions namely Central America. The pathogenesis and risk factors remain unclear.
Clinical case: We report a five years Old Portuguese boy, previously healthy, brought
to the Emergency Department with epistaxis, without other accompanying signs or
symptoms. The Otorhinolaryngologist (ORL) performed rhinoscopy and identified an
intranasal bleeding mass. The MRI revealed an intranasal mass with extension to the
ethmoid bone sinus, and performed biopsy. The histopathology was vital, making the
diagnosis of Rhinoscleroma. The child had traveled abroad for the first time on
vacations a year before to Dominican Republic. The bacteriologic exam identified a
Klebsiella spp. sensible to the association of amoxicillin and clavulanic acid. Blood test performed excluded association of immunodeficiency. Since it’s a rare disease genetic study are under course. Monthly evaluation by ORL and pediatrician was performed which documented progressive reduction until total disappearing of the macro and microscopic lesion, and negative bacteriologic exam. Six months of antibiotic therapy were completed without any known secondary effects. The child remained
asymptomatic up to the last visit, 3 months following treatment and has shown no
evidence of recurrence.
Conclusion: Globalization and free transit of people to areas far from origin countries
here some rare diseases are endemic brings a new challenge to modern medicine.
Sometimes vacations bring more than memories