Eosinophilic granuloma
Objectives- Discuss the relationship and/ or nomenclature of eosinophilic
granuloma, Letterer-Siwe disease, Hand-Schuller-Christian disease,
histiocytosis X, and Langerhans-cell granulomatosis
- Discuss the bones most frequently affected by eosinophilic granuloma
- Discuss treatment of spine lesions (vertebra plana) associated with eosinophilic granuloma
Discussion
Bony and soft tissue lesions involving the histiocytosis compromise a confederation of disorders with a somewhat confusing terminology. A spectrum of severity is generally inferred, with eosinophilic granuloma being the most benign, Hand-Schuller-Christian being intermediate, and Letterer-Siwe as by far most serious. Children with Letterer-Siwe disease primarily have hepatosplenomegaly, pulmonary disease, and anemia, and are rarely seen by the orthopaedist. Children with Hand-Schuller-Christian disease also have hepatosplenomegaly in addition to exophthalmus, and diabetes insipidus secondary to cranial involvement. Eosinophilic granuloma is manifested radiographically by a punched out lesion of bone, generally without surrounding bony reaction. Since the pathology of all these disorders is characterized by a dominance of proliferative histiocytes, the conditions have been collectively labeled Histiocytosis X. Langerhans-cell granulomatosis is another term that seems to be used more frequently. The skull is most often affected, but the spine and ilium are also frequent sites of eosinophilic granuloma and the reason for orthopaedic attention. There seems to be no propensity for eosinophilic granuloma to porgressing into the more severe form of Langerhans-cell granulomatosis.
Traditionally, eosinophilic granuloma was treated by excision. There is now a convincing literature that spine lesions can be treated with immobilization. Intralesional needle aspiration followed by injection of corticosteroids has recently been reported with very favorable results. Other series usually include some children treated with radiotherapy or chemotherapy. Joint management with a pediatric oncologist is indicated for the most accurate diagnosis and effective treatment of this disorder, as it is likely that further cytogenetic analysis will help better delineate this class of disorders.
References
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May 16-19, 2012 in Denver, CO
