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Histiocytosis X is an immunological disorder of unknown cause leading to abnormal proliferation of the histiocytes, and may manifest with either localized proliferation or more extensive systemic involvement. Oral manifestations include swelling or ulceration of the mandibular or maxillary bone. The mandible is commonly affected. Progressive alveolar bone loss in young children may lead to precocious exfoliation of primary teeth. It can also affect adolescents and adults, and presents as pain and swelling of the jaw with loosening of teeth. Radiographically, the tooth has a “floating in air” appearance surrounded by radiolucent areas. Ulcerations may develop on the gingiva, palate and the floor of the mouth. Oral lesions may occur even without underlying bone destruction.

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