Multifocal head and neck amyloidosis as a diagnostic clue of systemic lupus erythematosus (SLE)

Abstract

Rationale:Amyloidosis accounts for 2% of head and neck tumors. Amyloidosis that develops in the head and neck region is localized amyloidosis. Multifocal amyloidosis in the head and neck region is extremely rare.Patient concerns:The patient presented to the clinic of otolaryngology with nasal obstruction, anosmia and left neck mass for several months.Diagnosis:A left nasopharynx tumor was revealed under nasopharyngeal scope. Eosinophilic, proteinaceous material was revealed under a pathology scope in the nasopharynx tissue and neck tumor. Congo red staining demonstrated pale congophilic amorphous material with apple-green birefringence under cross-polarized light, and multifocal amyloidosis was diagnosed. Amyloidosis secondary to systemic lupus erythematosus (SLE) was confirmed after a series of investigations.Interventions:The patient underwent local excision for multifocal amyloidosis without following management. To control underlying SLE, the patient accepted steroid pulse therapy and immunosuppressants. The patient eventually achieved disease remission.Outcomes:During the 6 months of follow-up in the outpatient department of otolaryngology and rheumatology, complications, recurrence of nasopharyngeal amyloidosis, and SLE flare-up were not observed.Lessons:Head and neck amyloidosis involving the nasopharynx is a rare presentation of this disease. Head and neck multifocal amyloidosis should be taken as a hint of systemic disease. In head and neck amyloidosis, a comprehensive survey should be performed to clarify the underlying disease predisposing to amyloidosis and organ involvement.

Clinicopathological Features of Patients Nominated for Head and Neck Biopsies: A One-Year Series

“…Many benign head and neck lesions were widely reported. Some of these tumors are potentially neoplastic and others are non-neoplastic [ 4 , 5 ]. Consequently, the overall management of any head and neck tumor is deemed important for better patient outcomes.…”

Biclonal gammopathy of undetermined significance in a patient with systemic lupus erythematosus and antiphospholipid syndrome

“…This is particularly true in a condition such as SLE, in which there is an increased risk of hematological, pancreatic and lung tumors (8). It could also be an expression of systemic light chain amyloidosis (AL), a rare but known association in SLE (9). In AL, there is a prevalence of light chains λ over the k ones, like in our patient, as opposed to what happens in healthy subjects.…”

Amyloidosis in the nasopharynx and larynx: a case report

“…The diagnosis is based on biopsy and Congo red staining. 8 The nascent polypeptide chains of amyloid consist of ribosomes and are arranged in alpha or beta chains. Moreover, the body does not have an enzyme to digest the β-fold structure of macromolecules; therefore, the structure of the β-amyloid protein can cause amyloid amyloidosis to have polar birefringence characteristics, exhibiting apple-green birefringence under a polarizing microscope.…”