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October 1980

Localized Eosinophilic Granuloma (Langerhans' Cell Histiocytosis) of the Orbital Frontal Bone

Author Affiliations

From the Department of Ophthalmology, New York Hospital-Cornell University Medical Center and Manhattan Eye, Ear and Throat Hospital, New York (Drs Jakobiec and Iwamoto); the Edward S. Harkness Eye Institute, New York (Dr Trokel); Fondation Ophtalmologique A. de Rothschild and Hôpital Trousseau, Paris (Dr Aron-Rosa); and the Department of Radiology, Hôpital Bicêtre, Paris (Dr Doyon).

Arch Ophthalmol. 1980;98(10):1814-1820. doi:10.1001/archopht.1980.01020040666015

• Four patients (aged 5, 8, 14, and 51 years at initial manifestation) with isolated eosinophilic granulomas of the orbital frontal bone displayed short symptomatic periods (two weeks to three months) and some combination of erythema of the lids, a soft, palpable anterior orbital mass, periorbital pain, and osteolytic bone lesions on roentgenography. The bone lesions roentgenographically exhibited irregular, serrated, and sclerotic margins, distinguishing them from the more oval appearance of dermoid cysts. Electron microscopy performed in one case disclosed the presence of Langerhans' granules in the cytoplasm of the histiocytes, indicating that the orbital disease is a mild form of "histiocytosis X" and a benign proliferation of a specific kind of histiocyte—the Langerhans' cell. On follow-up (two to 20 years), after incomplete curettage of two lesions coupled with low doses of postoperative radiotherapy, there was reconstitution of the bone defects, whereas more extensive surgery performed on the other two patients resulted in permanent but subclinical bone defects.

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