Localized Amyloidosis of the Nasal Mucosa: A Case Report and Review of the Literature

Amyloidosis is a disorder of protein folding in which various proteins automatically aggregate into a highly abnormal fibrillar conformation. Amyloidosis is classified into systemic and localized forms depending on whether the abnormal proteins deposited in several different organs or only a single organ. In localized amyloidosis of the head and neck regions, laryngeal amyloidosis is common; however, localized amyloidosis of the nose is extremely rare. We herein report a case of localized amyloidosis of the nose and review the relevant literature on localized sinonasal amyloidosis. A 41-year-old man presented with a history of severe nasal obstruction, which had persisted for two decades. Nasal endoscopy and imaging studies showed extensive thickening of the bilateral nasal mucosa and diffuse submucosal deposition of calcification. After histopathological and systemic examinations, he was diagnosed with localized amyloidosis of the nasal mucosa. Septoplasty and bilateral inferior turbinoplasty, which consisted of mucosal resection using an ultrasonic bone curette, was performed and his symptoms markedly improved. Localized sinonasal amyloidosis has a good prognosis and surgical resection should be selected as a first-line treatment; however, clinicians should recognize the high probability of recurrence.

Extremely Rare Intraoral Presentation of Localized Amyloidosis

Purpose. To present an extremely rare case of localized amyloidosis of the mucosa of the maxillary alveolar ridge. Case Report. A 71-year-old man was referred to the Department of Oral Surgery, School of Dental Medicine, University of Zagreb, for a persistent nodular formation in the edentulous ridge of the anterior maxillary region. The nodular formation had a reddish color, solid consistency, and an approximate size of 40 × 15 mm . Orthopantomographic imaging excluded bone resorption and defects. Histopathological assessments of the biopsy specimen showed that the stroma was occupied by a multiplied, partially hyalinized connective tissue. The samples were subsequently stained with Congo red, and collagen accumulation under polarized light showed an apple-green birefringence indicating amyloid. Subsequently, the nodular formation was completely excised and a maxillary total denture was made. The patient showed normal tissue healing with no sign of recurrence at a follow-up assessment 1.5 years after the procedure. Conclusion. This is only the third reported case of localized amyloidosis of the alveolar ridge mucosa. Histopathological analysis is the first step for diagnosis, but systemic tests, blood counts, urinalysis, bone marrow biopsy, electrocardiography, and digestive endoscopy are recommended to rule out a systemic disease.

A clinical study of primary cutaneous localized amyloidosis

<p class="abstract"><strong>Background:</strong> Amyloidosis refers to abnormal extracellular tissue deposition of one of the biochemically unrelated proteins that share certain characteristic staining properties such as apple green birefringence of congo red stained preparations under polarized light. Amyloid deposition may occur in many organs of the body (systemic amyloidosis) or may be restricted to one tissue site (localized amyloidosis).</p><p class="abstract"><strong>Methods:</strong> Observational study was conducted in the Department of Dermatology, Narayana Medical College and Hospital, Nellore. In this study of 70 patients a complete history and clinical data was taken including details of presenting complaints, age, site, duration, course of the disease was recorded. Special importance was given to the friction history using different scrubs, family history and photo exposure.<strong></strong></p><p class="abstract"><strong>Results:</strong> Out of 70 patients of cutaneous amyloidosis 55 patients (78.6%) were of macular amyloidosis. Male:female ratio was 1:2.3. Majority of the patients were housewives (42.8%) and 28.5% of agriculturists. Majority of the patients (40) with cutaneous amyloidosis had disease duration between 2-5 years. In 70 patients of cutaneous amyloidosis 64.3% were symptomatic. 92% patients give history of using scrub. 50% of patients had history of photo exposure. Positive family history was seen in 7 patients (10%) and most common site involved was extensor aspect of arm.</p><p class="abstract"><strong>Conclusions:</strong> From our study it can be concluded that cutaneous amyloidosis is more common among females, most patients are symptomatic, majority have history of using scrub and photo exposure and most common site involved is extensor aspect of arm.</p><p class="abstract"> </p>