This article has Open Peer Review reports available.
Angiolymphoid hyperplasia with eosinophilia occurring in bilateral eyelids
© Ueda et al.; licensee BioMed Central Ltd. 2013
Received: 31 October 2012
Accepted: 23 July 2013
Published: 6 August 2013
Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign lesion, primarily occurring in the head and neck. ALHE arising from the ocular adnexa is rare, and the bilateral presentation is especially rare in the eyelids.
A 64-year-old Japanese man presented with tearing. Multiple nodules, approximately 5 mm in size, were observed in bilateral upper and lower eyelids. Surgical excisions of the both eyelids masses were performed. Histopathological examination of the excised masses demonstrated proliferated blood vessels lined by plump endothelial cells together with a lymphoid and eosinopilic infiltrate, compatible with a diagnosis of ALHE. Flow cytometry studies showed that the mass consisted of mostly CD3-positive cells. During two-year follow-up, no recurrence of the mass was observed and the patient had no subjective symptom of tearing
ALHE may occur in the bilateral eyelids. The cause of ALHE remains uncertain, but our results of flow cytemetry suggest that T cells are related to the pathogenesis of this disease.
KeywordsAngiolymphoid hyperplasia with eosinophilia Kimura’s disease Flow cytometry
Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, poorly understood, benign slow-growing lesion . The most common lesions seen in this entity are dermal or subcutaneous nodules of the face, scalp, neck and ears. Involvement of the ocular adnexa is rare compared with lesions of other sites [2, 3]. The reported cases of ALHE occurring in the eyelid were unilateral [4, 5].
We report a case of ALHE occurring in bilateral eyelids and discuss the clinical, histopathological and flow cytemetric features.
Subsequently the right eyelid mass was surgically excised. Residual lesions were removed piece by piece. At the end of surgery, triamcinolone acetonide was injected again into the eyelid. Although the patient refused additional surgery at first, one year after the initial operation, excision of left upper eyelid masses was performed in the same manner as the right eyelid mass. Histopathological and flow cytometric findings were the same as the right mass. During follow-up of two years, although some lesions remained unexcised, no recurrence of the excised masses was observed and the patient had no subjective symptom of tearing.
ALHE arising from the ocular adnexa is rare. ALHE occurring in eyelids which presented as bilateral multiple nodules has never been reported. The diagnosis of Kimura’s disease should be kept in mind in patients with a subcutaneous mass in the periocular region . ALHE and Kimura’s disease are known to have similar clinical, laboratory and histopathological findings. Whether the two diseases are distinct or variations of the same disease have been discussed for many years. The main distinguishing feature between these two diseases is histopathological findings. Both diseases have an infiltrate of mostly lymphocytes and eosinophils, but ALHE is characterized by proliferating blood vessels lined by plump epithelioid endothelial cells, compared with the flat endothelial cells in Kimura’s disease. Moreover, elevations of blood eosinophils and serum immunoglobulin E in laboratory examinations characterize Kimura’s disease. Our case was diagnosed as ALHE instead of Kimura’s disease based on the differential features. Recently many studies have concluded that the two are distinct disorders .
The cause of ALHE remains uncertain. Our flow cytemetric study demonstrated that the mass was consisted of CD3-positive cells, but there was no TCR rearrangement. These findings suggest that the pathogenesis of ALHE might be a T cell reactive process. In some previous reports, a history of trauma or evidence of associated damage or rupture of blood vessels was present in many cases. Therefore, some authors considered ALHE as a reactive process. Moreover, the bilateral presentation in our case also suggests that there is a reactive process. On the other hand, others considered these lesions as neoplastic . Previous papers have reported that these masses are consisted of mostly CD3-positive T cells  with monoclonality .
Complete surgical excision seems to be the most effective treatment . However, facial ALHE is often impossible to remove completely. The recurrence rate after surgical excision is approximately 33% , and is especially higher after a partial removal . Other treatments such as injection of corticosteroids and radiotherapy are generally not successful . Our patient also resisted local steroid treatment although we tried several injections, but there was no recurrence in two years following surgical excision.
ALHE may occur in the bilateral eyelids, albeit rarely. It is necessary to include ALHE in the differential diagnosis of an eyelid mass.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Series Editor of this journal.
The authors would like to thank Dr. Kusama Hiroshi from the Department of Anatomic Pathology at the Toda Central Medical Laboratory for assistance of pathological diagnosis.
- Wells GC, Whimster IW: Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol. 1969, 81: 1-14.View ArticlePubMedGoogle Scholar
- Esmaili DD, Chang EL, O'Hearn TM, Smith RE, Rao NA: Simultaneous presentation of Kimura disease and angiolymphoid hyperplasia with eosinophilia. Ophthal Plast Reconstr Surg. 2008, 24: 310-311. 10.1097/IOP.0b013e31817e9bba.View ArticlePubMedGoogle Scholar
- Hidayat AA, Cameron JD, Font RL, Zimmerman LE: Angiolymphoid hyperplasia with eosinophilia (Kimura's disease) of the orbit and ocular adnexa. Am J Ophthalmol. 1983, 96: 176-189.View ArticlePubMedGoogle Scholar
- Thompson MJ, Whitehead J, Gunkel JL, Kulkarni AD: Angiolymphoid hyperplasia with eosinophilia affecting the eyelids. Arch Ophthalmol. 2007, 125: 987-10.1001/archopht.125.7.987.View ArticlePubMedGoogle Scholar
- Kahana A, Lee BJ, Flint A, Elner VM: Angiolymphoid hyperplasia with eosinophilia affecting the eyelids. Arch Ophthalmol. 2012, 130: 1909-1912.View ArticleGoogle Scholar
- Seregard S: Angiolymphoid hyperplasia with eosinophilia should not be confused with Kimura's disease. Acta Ophthalmol Scand. 2001, 79: 91-93. 10.1034/j.1600-0420.2001.079001091.x.View ArticlePubMedGoogle Scholar
- Chun SI, Ji HG: Kimura's disease and angiolymphoid hyperplasia with eosinophilia: clinical and histopathologic differences. J Am Acad Dermatol. 1992, 27: 954-958. 10.1016/0190-9622(92)70293-O.View ArticlePubMedGoogle Scholar
- Helander SD, Peters MS, Kuo TT, Su WP: Kimura's disease and angiolymphoid hyperplasia with eosinophilia: new observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins. J Cutan Pathol. 1995, 22: 319-326. 10.1111/j.1600-0560.1995.tb01414.x.View ArticlePubMedGoogle Scholar
- Chen JF, Gao HW, Wu BY, Tsai WC, Chiang CP: Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: a rare case report with molecular evidence of T-cell clonality. J Dermatol. 2010, 37: 355-359. 10.1111/j.1346-8138.2010.00818.x.View ArticlePubMedGoogle Scholar
- McEachren TM, Brownstein S, Jordan DR, Montpetit VA, Font RL: Epithelioid hemangioma of the orbit. Ophthalmology. 2000, 107: 806-810. 10.1016/S0161-6420(00)00011-7.View ArticlePubMedGoogle Scholar
- Sanchez-Acosta A, Moreno-Arredondo D, Rubio-Solornio RI, Rodriguez-Martinez HA, Rodriguez-Reyes AA: Angiolymphoid hyperplasia with eosinophilia of the lacrimal gland: a case report. Orbit. 2008, 27: 195-198. 10.1080/01676830701804099.View ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/13/38/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.