Ricerc@Sapienza

Amyloidoses are heterogeneous diseases that result from the deposition of toxic insoluble β-sheet fibrillar protein aggregates in different tissues. The cascade of molecular events leading to amyloidoses and to the related clinical manifestations is not completely understood. Nevertheless, it is known that tissue damage associated to this disease involves alteration of tissue architecture, interaction with cell surface receptors, inflammation elicited by the amyloid protein deposition, oxidative stress, and apoptosis.

Isolated primary cutaneous amyloidosis (PCA) of the external ear is extremely rare. We describe the case of a 65-year-old woman presenting with itching within the left external auditory canal (EAC). Otoscopy revealed a 3 mm whitish lesion involving the cartilaginous portion of the left EAC. The lesion was excised. Histological and immunohistochemical features were consistent with keratinic amyloidosis. A clinical workup was negative for systemic amyloidosis. As far as we know, only nine cases of PCA exclusively involving the EAC have been reported.