Langerhans cell histiocytosis with orbital involvement

T. R. Kramer, R. J. Noecker, J. M. Miller, L. C. Clark

Research output: Contribution to journalArticle

30 Scopus citations

Abstract

PURPOSE: To review three cases of Langerhans cell histiocytosis with orbital involvement that represent a significantly excessive incidence of this rare disease in one community. Current diagnostic criteria and therapeutic modalities related to Langerhans cell histiocytosis are reviewed. METHODS: Case reports. We present clinical, radiologic, histopathologic, and epidemiologic information on three patients with Langerhans cell histiocytosis. RESULTS: All three children, born within 18 months of one another, manifested rapidly progressive unilateral proptosis at age 2 years. By computed tomography, all had moderately enhancing lesions with involvement of the sphenoid bone and lateral orbit as well as the temporal lobe of the brain. All patients were treated with a combination of vincristine and prednisone, with variable resolution of their lesions. The occurrence of three cases in children born in Nogales, Arizona/Mexico, suggests an incidence rate of 40 per million, which is approximately 26 times the expected rate (P = .0001). CONCLUSIONS: The extraordinarily high incidence and the concentration of cases in both time and space of this cluster implies that Langerhans cell histiocytosis may be a sentinel disease for unusual environmental exposures.

Original languageEnglish (US)
Pages (from-to)814-824
Number of pages11
JournalAmerican Journal of Ophthalmology
Volume124
Issue number6
DOIs
StatePublished - Jan 1 1997

ASJC Scopus subject areas

  • Ophthalmology

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