Int Arch Otorhinolaryngol 2014; 18 - a2482
DOI: 10.1055/s-0034-1388893

Systemic Amyloidosis: An Otorhinolaryngology Case

Amanda Lucas da Costa 1, Carolina Fischer Becker 1, Gabriel Kuhl 1, João Augusto Bergamaschi 1, Luíse Sgarabotto Pezzin 1, Luisi Rabaioli 1
  • 1Hospital de Clínicas de Porto Alegre

Introduction: Amyloidosis is a disease of extracellular deposition of insoluble protein light chain forming amyloid fibrils sheet configuration with B pregueada pentraxin serum amyloid (APS). These substances can be deposited in any organ or tissue. When this occurs in the nasopharyngeal and laryngeal regions, it is an extremely rare event, especially if associated with systemic amyloidosis. Generally, the disease presents localized and not systemic, causing lumiais obstructions in the affected organs. The diagnosis is confirmed by pathological examination with Congo red staining.

Case Report: A previously healthy, 52-year-old female patient, with signs of bilateral swelling of the hands and tingling fingers, misdiagnosed with carpal tunnel syndrome, received surgical treatment without improvement of symptoms. In the evolution of the framework presented with petechiae and ecchymosis, dysphonia, macroglossia, and lip swelling with progressive worsening, the patient arrives at the Hospital de Clinicas de Porto Alegre where the investigation is initiated and the diagnosis of cutaneous amyloidosis confirmed by skin biopsy.

Discussion: The pathological deposition of amyloid fibrils leads to tissue dysfunction manifesting clinically according to the organs involved, the most common presentations macroglossia, restrictive cardiomyopathy amyloid polyneuropathy, and nephropathy. Because of the multiplicity of manifestations, the diagnostic approach is often a clinical challenge. The diagnosis is confirmed by examination anatomopatologico tissue biopsy.