難治性耳漏を初発症状としたランゲルハンス細胞組織球症例

概要

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Langerhans cell histiocytosis (LCH) involves the proliferation and accumulation of Langerhans cells at various sites and presents with various symptoms, depending on the organ or organs invaded. A 2-year-old boy with LCH was treated at our hospital. Fever, head rash, bilateral chronic otorrhea unresponsive to antibiotics, and left exophthalmos were observed at our first examination. The CT scan showed destruction of the skull bone, zygomatic bone and vertebrae, and masses within these lesions. No bony defect was detectable in the middle ear, but a mass was present in the external auditory meatus. Skull X-rays revealed characteristic "punched-out" lesions. We confirmed the diagnosis of LCH with a biopsy of the head mass and the external auditory meatus mass. Immunohistology revealed positive staining of the lesional cells with CD1a and Langerin (CD207). The final diagnosis was multisystem LCH (MS-LCH). The child was placed on the chemotherapeutic regimen recommended by the Histiocyte Society.