Twenty percent of patients do not exhibit any cutaneous signs of disease and present solely with varying systemic complaints. The most common extracutaneous form of LCH, formerly designated eosinophilic granuloma, is now called single-system unifocal bone disease. Children present with a painless to slightly tender soft tissue swelling overlying the bony area of involvement, most commonly the calvarium. Palpation of the swelling reveals the fluctuant nature of the soft tissue distention, and in some cases the defect in the underlying bone can be felt. Plain radiographs can help delineate the extent of disease. If one area of bony involvement is found, a skeletal survey should be performed to evaluate for other silent bony lesions, which can occur in up to 15% of cases. The involved bone has a radiolucent appearance that is sharply demarcated from the surrounding bone. Bony involvement has been described to occur in almost every bone in the body. Most cases are inconsequential, but if the involvement affects a critical portion of the spine, the possibility of weakening of the joint and potential fracture could have life-threatening implications. The term “floating teeth” has been used to describe the finding of radiolucent aspects of the mandible that give the appearance that the teeth are floating without the support of the underlying bone.
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