후두를 침범한 파종성 황색종 1예

Abstract

Xanthoma disseminatum is a rare benign, normolipemic mucocutaneous xanthomatosis resulting due to the proliferation of non-Langerhans cell histiocytes. Xanthoma disseminatum typically involves the skin, particularly the flexor skin folds. Mucocutaneous involvement develops in 40-60% of patients, most commonly affecting the oropharynx, larynx, or corneae and conjunctivae. Xanthoma disseminatum may also manifest in the central nervous system. While the natural history of xanthoma disseminatum is usually benign, lesions in critical anatomical locations may result in morbidity and mortality. Curative therapy is lacking. We report a case with laryngeal involvement in a female patient. In this patient, xanthomas involved oropharynx and larynx mucosa. Dyspnea occurred due to epiglottis lesion. Tracheotomy was performed, and maintenance of breathing and decanulation were possible after epiglottectomy.