Localized amyloidosis of the nasal and paranasal mucosa: a rare pathology





Introduction


Amyloidosis is a rare condition characterized by the deposition of abnormal protein filaments into extracellular tissue. We present a patient with localized amyloidosis of the nasal and paranasal sinus mucosa and discuss the diagnostic challenges related to making the correct diagnosis.





Case report


The patient is a 55-year-old woman with a history of chronic sinusitis, rheumatoid arthritis, and monoclonal gammopathy of undetermined significance who presented with severe malaise, diffuse arthralgias, persistent nasal congestion, and elevated erythrocyte sedimentation rate. Computed tomographic scan of the sinuses was significant for an opacification of the left middle meatus and mucosal inflammation in all sinuses. A vasculitic process was considered, and an otolaryngologist was consulted for a nasal biopsy. Endoscopic examination demonstrated a nasal polyp arising from the left middle meatus. Biopsy of the mass was performed, and histopathologic examination revealed tissue consistent with an inflammatory nasal polyp.


The patient’s symptoms worsened, and she underwent operative biopsies of the right and left maxillary sinuses, nasal septum, and bilateral inferior turbinates. Histopathologic examination of all specimens revealed deposits of amorphous eosinophilic material involving vessels and lamina propria, consistent with amyloid ( Fig. 1 ). Congo red staining of the specimens revealed apple-green birefringence with polarized microscopy, which confirmed the diagnosis of amyloidosis ( Fig. 2 ). Reexamination of the initial biopsy by histology and Congo red stain failed to demonstrate amyloid. Aspiration biopsy of subcutaneous abdominal fat failed to reveal amyloid making systemic amyloidosis unlikely. The patient’s systemic symptoms were attributed to a rheumatoid arthritis flare in conjunction with a polymyalgia rheumatica–like syndrome, and she improved with steroid treatment.




Fig. 1


Respiratory epithelium with pink amorphous deposits in the lamina propria (hematoxylin-eosin stain, magnification ×40).



Fig. 2


Congo red stain with apple green birefringence of amyloid deposits when observed by polarizing microscope (Congo red stain, original magnification ×40).





Case report


The patient is a 55-year-old woman with a history of chronic sinusitis, rheumatoid arthritis, and monoclonal gammopathy of undetermined significance who presented with severe malaise, diffuse arthralgias, persistent nasal congestion, and elevated erythrocyte sedimentation rate. Computed tomographic scan of the sinuses was significant for an opacification of the left middle meatus and mucosal inflammation in all sinuses. A vasculitic process was considered, and an otolaryngologist was consulted for a nasal biopsy. Endoscopic examination demonstrated a nasal polyp arising from the left middle meatus. Biopsy of the mass was performed, and histopathologic examination revealed tissue consistent with an inflammatory nasal polyp.


The patient’s symptoms worsened, and she underwent operative biopsies of the right and left maxillary sinuses, nasal septum, and bilateral inferior turbinates. Histopathologic examination of all specimens revealed deposits of amorphous eosinophilic material involving vessels and lamina propria, consistent with amyloid ( Fig. 1 ). Congo red staining of the specimens revealed apple-green birefringence with polarized microscopy, which confirmed the diagnosis of amyloidosis ( Fig. 2 ). Reexamination of the initial biopsy by histology and Congo red stain failed to demonstrate amyloid. Aspiration biopsy of subcutaneous abdominal fat failed to reveal amyloid making systemic amyloidosis unlikely. The patient’s systemic symptoms were attributed to a rheumatoid arthritis flare in conjunction with a polymyalgia rheumatica–like syndrome, and she improved with steroid treatment.


Aug 25, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on Localized amyloidosis of the nasal and paranasal mucosa: a rare pathology

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