Unusual clinical presentation of amyloidosis: Bilateral stenosis of the external auditory canal, hoarseness and a rapid course of cutaneous lesions

Abstract

We report a patient with multiple myeloma associated with primary systemic amyloidosis who had a rapid evolution and a very unusual form of presentation. The association of amyloidosis in patients with multiple myeloma is 15%, and clinically evident mucocutaneous involvement occurs in up to 40% of patients.

Image Gallery: Cutaneous lesions in the external auditory canal causing hearing loss

“…In this case, the amyloid light‐chain amyloidosis was the first manifestation of multiple myeloma. Its presentation as multiple exophytic nodules occluding the EAC is unique, rather than the diffuse skin thickening of the EAC reported in previous cases …”

Post-inflammatory acquired atresia of the external auditory canal

“…Histopathological studies taken from the auditory canal were suggestive of pyoderma gangrenosum, a non-specific finding and a diagnosis of exclusion that could occur secondary to chronic systemic inflammatory diseases (ulcerative colitis, Crohn’s disease, rheumatoid arthritis, vasculitic disorders) and myeloproliferative disorders. On the other hand, acquired stenosis and atresia of the EAC have also been reported secondary to amyloidosis and fibrous dysplasia of the temporal bone ( Álvarez-Ruiz et al., 2007 ; Jethanamest and Roehm, 2011 ). Nonetheless, diffuse canal involvement occurred in these cases, in contrast to medial canal involvement that is classically reported in medial meatal fibrosis.…”

Amiloidosis asociada a diálisis en el conducto auditivo externo, presentación de un caso clínico y revisión de la literatura

“…Pocos casos de amiloidosis han sido reportados en el área otorrinolaringológica, siendo los lugares más descritos la laringe y la base de la lengua 6 . También se han descrito depósitos de amiloide en nasofaringe, cavidades paranasales y parótida [7][8][9][10][11][12][13][14][15] . No obstante las manifestaciones otológicas son extremadamente raras 8 .…”

“…The present case involved a 56 years old man who had been on hemodialysis for 36 years with bilateral amyloidosis of the external auditory canal. Only 13 cases of amyloidosis in the ear canal, including the present case have been reported in the literature [6][7][8][9][10][11][12][13][14] . This is the third reported case of dialysis related amyloidosis of the ear canal 6,10 .…”