Langerhans cell histiocytosis with orbital involvement
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Published on: September 1, 2018
T R Kramer1, R J Noecker, J M Miller
1Department of Ophthalmology, University of Arizona Health Sciences Center, Tucson 85719, USA. TKramer@eyes.Arizona.edu
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.
