73 • Vol 8 • January 2006 Localized Laryngeal Amyloidosis Amyloidosis is characterized by the extra- cellular deposition of abnormal insoluble fibrillar proteins in organs and tissues. The amyloid protein takes up Congo red stain and exhibits an apple-green birefringence under polarized microscopy [1]. Amyloidosis is usually a systemic disease with multiorgan involvement and a grave prognosis. Thus, detection of amyloid protein in any tissue usually leads to a thorough search for systemic involvement. Nevertheless, focal forms of amyloidosis with solitary organs but no systemic involvement rarely occur. The latter forms have a much better progno- sis [1,2]. We describe here a patient with recurrent laryngeal amyloidosis without systemic involvement. Patient Description A 63 year old woman with a 3 month history of progressive dyspnea on exer- tion and an episodic non-productive cough was admitted. Her medical history included well-controlled hypertension, migraine and parathyroidectomy due to adenoma. On examination, inspiratory stridor was present while at rest. The stridor increased after mild exertion. There was no change in her voice. The rest of the examination was normal. Rou- tine laboratory tests including erythrocyte sedimentation rate, urinalysis and chest X-ray were all normal. Direct laryngoscopy demonstrated an irregular subglottic lesion that originated immediately under the true vocal cords and descended 3 cm to the subglottic area. Biopsy from the lesion disclosed chronic inflammation, fibrosis and atypia of epithelial cells but no signs of malignancy. There was prominent sedimentation of a homogenous eosino- philic material with a typical apple-green birefringence under polarizing microscope after staining with Congo red. Stain for AA amyloid was negative. Debulking of the lesion was performed to relieve mechanical airflow obstruction, with complete resolution of symptoms. An ex- tensive workup for systemic amyloidosis including serum and urine electrophore- sis, bone marrow biopsy, subcutaneous fat aspiration, cardiac echo, bronchos- copy, chest computed tomography and abdominal ultrasound were all normal. Serum beta-2 microglobulin was mildly elevated at 3259 ng/ml (normal 0–1900). The patient was asymptomatic for 1 year follow- ing the operation, after which her dyspnea recurred accompanied by a mild hoarseness. Concomitantly, the level of serum β 2-microglobu- lin was further elevated (4453 ng/ml). Laryngo- scopy revealed bilateral subglotic submucosal lesion compatible with a mild recurrence of her laryngeal amyloidosis that did not require re- operation. Two months later, the patient developed severe dyspnea with stridor. Repeated laryngoscopy demonstrated severe subglotic stenosis [Figure]. At that time, extensive workup was again nega- tive for systemic disease. The patient was re-operated using a CO 2 Laser. Laryngeal tissue obtained was infiltrated with a Congo-red stained material. Following the second operation a clinical improvement was observed although residual exertional stridor persisted. Similarly, the β2-mi- croglobulin levels declined but remained elevated (3200 ng/ml). Four months later the patient underwent tracheostomy due to another local recurrence. Eighteen Exertion Dyspnea and Stridor: an Unusual Presentation of Localized Laryngeal Amyloidosis David Ergas MD 1 , Yigal Abramowitz MD 1 , Yonatan Lahav MD 2 , Doron Halperin MD 2 and Zev Moshe Sthoeger MD 1 Departments of 1 Medicine B and 2 Ear/Nose/Throat, Kaplan Medical Center, Rehovot, Israel Affiliated to Hebrew University-Hadassah Medical School, Jerusalem, Israel Key words: laryngeal amyloidosis, stridor, exertion dyspnea, localized amyloidosis IMAJ 2006;8:73–74 Direct laryngoscopy before re-operation shows recurrent disease (laryngeal amyloidosis). Immediately beneath the vocal cord there is bilateral submucosal encroachment on the lumen, leaving only a minute orifice. Case Communications