Korean J Otorhinolaryngol-Head Neck Surg.  2016 Oct;59(10):738-741. 10.3342/kjorl-hns.2016.59.10.738.

A Case of Erdheim-Chester Disease with Laryngeal Involvement

• Park JS
• Moon MG
• Lee JH
• Lee SH

• KMID: 2356328
• DOI: http://doi.org/10.3342/kjorl-hns.2016.59.10.738

Abstract

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis with multi-organ involvement. Bones, cardiovascular system, central nervous system, kidney, skin and many different organs can be involved but laryngeal involvement has not been reported in literatures. The diagnosis of ECD was based on clinical manifestations and immunohistochemical findings including CD68(+), CD1a(-) and S100(-). Currently, Interferon-a is the most extensively studied agent in the treatment of ECD and serves as the 1st line of treatment. Surgical resection of involved lesions can be tried but it leads to temporary improvement. A 60-year-old man visited with respiratory symptoms because of ECD with laryngeal involvement. We resected the obstructive lesion to relieve the symptoms. Tissue biopsy gave a diagnosis of ECD. As it is rare to encounter ECD involving the larynx, we report this case with a review of literatures.

Keyword

Epiglottis; Erdheim-Chester disease; Larynx; Non-Langerhans cell histiocytosis

MeSH Terms

Biopsy
Cardiovascular System
Central Nervous System
Diagnosis
Epiglottis
Erdheim-Chester Disease*
Histiocytosis
Humans
Kidney
Larynx
Middle Aged
Skin