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Author Notes:

Correspondence: Hans E. Grossniklaus, MD, Emory Eye Center, L.F. Montgomery Laboratory, BT 428, 1365 Clifton Road, Atlanta, Georgia 30322, USA; Phone: (+1) 404-778-4611; Fax: (+1) 404-778-4610; Email: ophtheg@emory.edu

Disclosures: The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article.

Subjects:

Research Funding:

This work was supported in part by an unrestricted departmental grant from Research to Prevent Blindness, Inc, NIH P30EY06360 (Hans E. Grossniklaus), and the German Research Foundation (DFG, Martina C. Herwig).

Keywords:

  • Eosinophilic granuloma
  • Langerhans cell histiocytosis (LCH)
  • Orbit

Langerhans Cell Histiocytosis of the Orbit

Tools:

Journal Title:

Survey of Ophthalmology

Volume:

Volume 58, Number 4

Publisher:

, Pages 330-340

Type of Work:

Article | Post-print: After Peer Review

Abstract:

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. Herein, we describe the clinical and histologic spectrum of LCH of the orbit on the basis of five cases. One patient exhibited multifocal unisystem disease, the other four patients presented with a localized process. The typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunostaining. Transmission electron microscopy exhibited characteristic intracytoplasmic Birbeck granules. The different ophthalmic manifestations of LCH and treatment strategies are reviewed in the context of previously reported cases. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH has to be included in the differential diagnosis in tumors of the ocular adnexae, in particular in young children.

Copyright information:

© 2013, Elsevier

This is an Open Access work distributed under the terms of the Creative Commons Attribution-NonCommerical-NoDerivs 3.0 Unported License (http://creativecommons.org/licenses/by-nc-nd/3.0/).

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