耳漏を反復したランゲルハンス細胞組織球症

Abstract

Langerhans' cell histiocytosis (LCH), formerly termed histiocytosis X, is a single or multisystem disease. It is characterized by the proliferation and accumulation of Langerhans' cells in the debmis and/or various extracutaneous sites. Skin involvement is a frequent manifestation in young children, and otitis externa is a common symptom.A 20 month-old boy presented with a chronic scalp and trunk rash, and bilateral chronic otorrhea unresponsive to oral antibiotics and topical antibiotic-steroid drops. A physical examination revealed swelling of the posterosuperior quadrant of the bilateral tympanic membranes, and an accumulation of white debris fixed to the tympanic membrane was also present bilaterally. A computed tomograph of the middle ear was almost normal, but showed a thickening of the bilateral tympanic membranes. An audiologic evaluation revealed moderate (-40dB) hearing loss on the right side, and total deafness on the left side. The tympanogram was flat bilaterally. A diagnosis of bilateral chronic persistent otitis externa with essential deafness on the left side was made.After a 6 month trial of conservative therapy, he suddenly suffered from a severe impairment in his respiration. A chest X-ray showed large patchy areas of water density throughout both lungs, and skull X-rays revealed characteristic “punched-out” lesions. Skin biopsies from the scalp and trunk revealed a large accumulation of Langerhans' cells in the lesion, and thus confirmed a diagnosis of LCH. Thereafter, the child was placed on a chemotherapeutic regimen consisting of vinblastine and prednisolone. In spite of intensive care for 3 weeks, he died due to total pulmonary dysfunction.LCH is now considered to be a rather non-malignant process. However, as in the current case, the prognosis is quite poor in children under 2 years who present with multi-system diseases and major organ dysfunction. If a physician experiences a case of bilateral chronic otorrhea unresponsive to oral antibiotics and topical antibiotic-steroid drops with a chronic skin rash, then he should consider the presence of systemic and/or local immune deficiency due to LCH. An early diagnosis of LCH with a skin biopsy, and then rapid treatment with intensive systemic regimes using vinblastine combined with corticosteroids would be recommended.