Disseminated Amyloidosis in Head and Neck - Literature Review and Discussion of an Unusual Case

Abstract

Primary amyloidosis of head and neck is an extremely rare event and there are few cases reported in the literature. Amyloidosis is a group of diseases that have as etiology the extracellular proteins deposition in many organs, causing architectural and functional damage to them. This article´s objective is to report a case of primary amyloidosis localized in skin, tongue, parotid and upper airways simultaneously in one patient and do a literature review on the topic. Patient W.K.Y. 72 years-old, from China, living in Brazil for 6 months, come out with vesicles in the head and neck about one year ago. The disease had been spreading to face and neck skin as well as lips, oral mucosa and tongue. Because of oral feeding impairment and dyspnoea, the patient was taken to an emergency service, where clinical investigation started. As previous treatment, It was reported the use of Chinese medicine, acupuncture and herbs. Patient had no diabetes, hypertension, Alzheimer’s disease or nephropathy. Also, he had no family history of any degenerative or infectious disease. Patient diagnosis was: primary amyloidosis with secondary rheumatoid component. For treatment, a prednisolone pulse therapy associated with colchicine was elected, causing regression edema of tongue, suprahyoid region, lips and parotid glands, likewise about 50% of skin neck vesicles disappeared Head and neck Amyloidosis occurs in 19% of cases and may affect the skin, ear, nasal cavity, oral cavity, parotid, pharynx and larynx. Larynx is the most commonly affected organ in this region, accounting for the largest number of case reports and case series in the literature, with treatment and well-established management. The diagnosis is made based on histopathologycal examination and biochemical characterization of amyloid type. The treatment is based on the surgical resection with variable recurrence rates, according to affected organ.