Oto-Rhino-Laryngology (ORL) tumor presentation in a case of systemic AA amyloidosis.

A 64-year-old male with no underlying disease presented with the development of multiple skin nodules, loss of sensation in the extremities, hoarseness, macroglossia, and pain in the oral cavity. Direct laryngoscopy showed nodules involving the oral cavity, oropharynx, supraglottic region, and vocal cords. Biopsy from skin nodules showed amyloid deposits staining with Congo red. Immunohistochemical staining was used for AA protein and was positive. Biopsy from the oral floor was also positive for amyloid. Oto-Rhino-Laryngology (ORL) involvement has been reported in approximately 40 percent of AL amyloidosis patients, but does not appear to be frequent in AA amyloidosis. Cutaneous manifestations in AA amyloidosis are rare, although cases with lesions presenting as purpura are reported occasionally; we are not aware of other cases of ORL nodular involvement in systemic AA.