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Brief Report |

Angiolymphoid Hyperplasia With Eosinophilia of the Orbit and Ocular Adnexa:  Report of 5 Cases

Amir A. Azari, MD1; Mozhgan R. Kanavi, MD2; Mark Lucarelli, MD1,2; Vivian Lee, MD1; Ashley M. Lundin, MD1; Heather D. Potter, MD1; Daniel M. Albert, MD1,3
[+] Author Affiliations
1Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison
2Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3McPherson Eye Research Institute, University of Wisconsin, Madison
JAMA Ophthalmol. 2014;132(5):633-636. doi:10.1001/jamaophthalmol.2013.8243.
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Importance  To report the clinical and histopathologic findings of ocular adnexal angiolymphoid hyperplasia with eosinophilia, an unusual but often misdiagnosed benign disorder.

Observations  The ophthalmologic findings of angiolymphoid hyperplasia with eosinophilia with ocular adnexal involvement are variable and include eyelid swelling, ptosis, proptosis, and loss of vision. Imaging studies typically reveal a well-circumscribed mass in the orbit. The condition may resemble other diseases that involve the orbit and ocular adnexal tissue, such as lymphoma, hemangioma, sarcoidosis, and dermoid cyst. Histopathologic analysis reveals marked vascular proliferation with an accompanying inflammation composed of numerous eosinophils, lymphocytes, and plasma cells.

Conclusions and Relevance  Angiolymphoid hyperplasia with eosinophilia is a rare disease that can affect the ocular adnexal tissue. The clinical presentation is often nonspecific; therefore, histopathologic studies are essential for diagnosis and subsequent management of this benign condition.

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Figure 1.
Clinical Presentation of the 5 Patients With Angiolymphoid Hyperplasia With Eosinophilia (ALHE)

A through E, Clinical photographs and radiologic findings in patients with orbital ALHE. The photographs show an anterior orbital mass that involves the left side with mild swelling and ptosis in patient 1 (A), patient 2 (B), patient 4 (D), and patient 5 (E). C, The computed tomogram in patient 3 shows an extraconal mass in the right orbit. E, In patient 5, there is marked involvement of the upper and lower eyelids.

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Figure 2.
Histopathologic Studies of Patients With Angiolymphoid Hyperplasia With Eosinophilia

A through E, Histologic examination with hematoxylin-eosin stain reveals inflammation marked by a large number of eosinophils admixed with lymphocytes and plasma cells in all patients. Vascular proliferation of the capillaries, arterioles, and venules are best seen in patient 3 (C) and patient 4 (D). The endothelial cells lining the vessels are histiocyte-like and contain intracytoplasmic vacuoles, which are depicted with arrowheads in patient 5 (E) but were present in all patients.

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