The frequency of laryngeal involvement in lipoid proteinosis patients

Abstract

Aim: Lipoid proteinosis (LP) is a rare autosomal recessive disorder that occurs with the accumulation of hyaline substance in oralcavity, laryngeal mucosa and skin. The aim of this study was to investigate the incidence of laryngeal involvement in patients withLP which have hoarseness and skin lesions, and the areas of hyaline substance accumulation in the larynx.Material and Methods: In this study, 21 patients who were admitted to our Dermatology outpatient clinic between January 2013 andDecember 2017 and diagnosed as LP with punch biopsy were evaluated. Laryngeal imaging was performed in otorhinolaryngologyclinic, and the frequency of larynx involvement and the areas of hyaline accumulation in the larynx were evaluated.Results: Twenty-one patients with hoarseness and diagnosed by skin biopsy as LP were included in the study. Thickening ofthe vocal cords in 21 patients (100%); thickening and hypertrophic changes of arytenoids in 13 patients (61.9%); thickening andhypertrophic changes of the interarytenoid area in 10 patients (47.6%); thickeningand hypertrophic changes of epiglottis in 3 (14.3%)patients; hypertrophic changes and thickening of the aryepiglottic fold in 3 patients (14.3%); thickening and hypertrophic changes ofventricular folds in 2 patients (9.5%) were observed.Conclusion: In our study, similar to the data in the literature, the pathological changes were most frequently seen in the vocal cordsand the least in the ventricle band, aryepiglottic fold and epiglottis. In order to better understand the laryngeal involvement and theareas of involvement in LP patients, studies with more patients are needed.