両側外耳道病変を初発とした多臓器型ランゲルハンス細胞組織球症の2例

抄録

Langerhans cell histiocytosis (LCH) is a disease characterized by the proliferation of Langerhans cells, one of the antigen-presenting cells, that can manifest in a variety of symptoms involving various parts of the body, such as the skin, bones, and internal organs. It is a relatively rare disease, occurring at an incidence of about five cases per million children. Although it is a rare disease, 39% of LCH patients have some kind of otologic symptoms and often visit otolaryngologists for otorrhea and external ear masses. In the present study, we report two cases of LCH in which the patients initially presented to otolaryngologists for external auditory canal symptoms and were later diagnosed as having multisystem LCH.

The prognosis of multisystem LCH is often poor, and the patients often suffer from sequelae. Therefore, early diagnosis and initiation of treatment are very important. Otolaryngologists encountering intractable otitis externa or masses in the external auditory canal in children should bear in mind the possibility of LCH and collaborate with pediatricians for biopsy and search for a systemic cause.