Korean J Otorhinolaryngol-Head Neck Surg.  2007 Nov;50(11):1065-1068.

Xanthoma Disseminatum, a Case with Laryngeal Involvement

• Jung E
• Rha KW
• Kim SK
• Choi HS

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.

Keyword

Xanthoma disseminatum; Epiglottis

MeSH Terms

Central Nervous System
Conjunctiva
Cornea
Dyspnea
Epiglottis
Female
Histiocytes
Histiocytosis, Non-Langerhans-Cell*
Humans
Larynx
Mortality
Mucous Membrane
Natural History
Oropharynx
Respiration
Skin
Tracheotomy
Xanthomatosis*