LCH has a variable course. The use of the nomenclature of eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe syndrome still plays a role in describing the disease, although there is significant overlap.
Eosinophilic granuloma (i.e., unifocal LCH) is characterized by the formation of solitary or multiple discrete nodules within bones and represents the majority of cases (60-80%). These lesions are typically found in children younger than 15 years. There is associated pain and tenderness with these lesions, and there may be difficulty with weight bearing (2).
Hand-Schuller-Christian disease (multifocal LCH) has similar bone "granulomas" along with other systemic manifestations. The skeletal anatomy of the head and neck is prominently involved. Mandibular defects include severe gingivitis, loss of mandibular height, and multiple loose teeth. The skull can have a "geographic skull" appearance on plain films secondary to multiple lesions. Involvement of orbital bones can result in changes in vision, and blindness can occur. Sellar involvement around the pituitary can lead to hypopituitarism, resulting in short stature and diabetes insipidus (2).
Otologic manifestations are particularly relevant to the otolaryngologist. Patients may present at any time during childhood. Mastoid disease may present in unifocal, multifocal, or systemic LCH. Patients typically present with otorrhea. The incidence of LCH ear disease (all types) is from 15% to 61%. It is usually unilateral, but bilateral disease may occur. The presence of polyps and granulation tissue in the external canal is highly suggestive of LCH. The middle ear is usually spared. Otitis externa can also be present. LCH can mimic cholesteatoma and should be kept in mind as a part of the differential of otorrhea, especially if bloody. A computed tomography (CT) scan typically shows aggressive lytic lesions similar to osteomyelitis, bone lymphoma, or sarcoma (4,5).
Letterer-Siwe disease (systemic LCH) is the rarest (approximately 10% of all LCH cases) and most severe form of LCH. Affected individuals are typically under the age of two years and present with diffuse eczema, draining ears, lymphadenopathy, and hepatosple-nomegaly. Failure to thrive, weight loss, and pancytopenia are also present and portend a poor prognosis (2).
Another manifestation of LCH is primary pulmonary LCH, which affects both children and adults. Pulmonary LCH has been associated with cigarette smoking in adults, and the course tends to be severe. The childhood form of pulmonary LCH appears to be less severe but more chronic in nature (6).
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