A Case of Erdheim-Chester Disease with Laryngeal Involvement.
10.3342/kjorl-hns.2016.59.10.738
- Author:
Jin Su PARK
1
;
Myung Gi MOON
;
Jae Hyuk LEE
;
Sang Hyuk LEE
Author Information
1. Department of Otorhinolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea. entlsh@hanmail.net
- Publication Type:Case Report
- Keywords:
Epiglottis;
Erdheim-Chester disease;
Larynx;
Non-Langerhans cell histiocytosis
- MeSH:
Biopsy;
Cardiovascular System;
Central Nervous System;
Diagnosis;
Epiglottis;
Erdheim-Chester Disease*;
Histiocytosis;
Humans;
Kidney;
Larynx;
Middle Aged;
Skin
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2016;59(10):738-741
- CountryRepublic of Korea
- Language:Korean
-
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.
