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Year : 2022  |  Volume : 10  |  Issue : 1  |  Page : 33-35

Kimura's disease involving the conjunctiva

1 Department of Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, PBMA's H. V. Desai Eye Hospital, Pune, Maharashtra, India
2 Department of Histopathology, Ruby Hall Clinic, Pune, Maharashtra, India

Date of Submission14-Jul-2020
Date of Decision10-Aug-2021
Date of Acceptance24-Aug-2021
Date of Web Publication3-Feb-2022

Correspondence Address:
Sonal P Yadav
Department of Ophthalmic Plastic Surgery and Ocular Oncology, PBMA's H. V. Desai Eye Hospital, Mohammed Wadi, Pune - 411 060, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcor.jcor_104_20

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A 50-year-old female presented with gradually increasing, pink nodular lesion on the temporal bulbar conjunctiva of the left eye, noticed in the past 15 days. She denied any history of trauma or similar episodes in the past or concurrent systemic illness. The lesion did not change after a 2-weeks long topical steroid course. It was then surgically excised. Histopathological analysis revealed lymphoid follicles with proliferating blood vessels lined up by plump endothelial cells with mixed infiltrate containing numerous eosinophils. Based on these findings, diagnosis of Kimura's disease (KD) was established. KD is a rare, chronic inflammatory disorder of possible allergic or autoimmune etiology which has been rarely described in conjunctiva.

Keywords: Conjunctiva, Kimura's disease, ocular surface, solitary conjunctival nodule

How to cite this article:
Yadav SP, Patil S, Puntambekar A, Deshpande R. Kimura's disease involving the conjunctiva. J Clin Ophthalmol Res 2022;10:33-5

How to cite this URL:
Yadav SP, Patil S, Puntambekar A, Deshpande R. Kimura's disease involving the conjunctiva. J Clin Ophthalmol Res [serial online] 2022 [cited 2022 May 29];10:33-5. Available from: https://www.jcor.in/text.asp?2022/10/1/33/337186

Kimura's disease (KD) is an uncommon chronic inflammatory disorder involving the subcutaneous tissues and lymph nodes in the head-and-neck region of young Asian males.[1],[2],[3] Involvement of orbit, eyelid, and extraocular muscle in KD is unusual and sparsely reported.[4],[5] We report a rare case of KD with the involvement of conjunctiva with its clinical and histological features. Informed consent was obtained for the use of her clinical photographs for academic and research purposes.

  Case Report Top

A 50-year-old female presented with a history of noticing a gradually growing reddish lesion on the temporal part of conjunctiva of her left eye for the past 15 days. She denied any history of antecedent trauma, history of similar episodes, or concurrent systemic illness. On slit-lamp examination, a 12 mm × 10 mm × 3 mm elevated subconjunctival nodular lesion in the superior-temporal quadrant of the left eye was noted [Figure 1]a. The nodule was not fixed to the underlying episclera and was away from the limbus [Figure 1]b. The rest of the ocular surface and anterior with posterior segment examination was unremarkable. Her best-corrected visual acuity was 20/20 with intraocular pressure of 16 mm of Hg in both the eyes. The examination of the right eye was within the normal limits. On physical examination, there was no palpable lymphadenopathy.
Figure 1: Clinical and histological features. (a and b) Left eye, 12 mm × 10 mm × 3 mm solitary conjunctival lesion in supero-temporal quadrant with congestion of overlying conjunctiva, (c) (H and E) low power view showing lymphoid hyperplasia, (d) (H and E) Intermediate power showing proliferating vessels lined by plump endothelial cells with mixed infiltrate with numerous eosinophils, and (e) (H and E) High power view showing prominent eosinophilic infiltrate

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Considering a presumed diagnosis of nodular episcleritis, she was prescribed a course of topical steroids. The complete blood count, erythrocyte sedimentation rate, C-reactive protein, and rheumatoid arthritis factor were within the normal range. On 2-weeks of follow-up, the lesion showed minimal change. She then underwent complete excision of the lesion under local anesthesia with direct closure of conjunctiva to achieve surface reconstruction. Intraoperatively, adhesions and fibrosis were noted in the surrounding tenon's capsule, probably secondary to topical steroid use. On gross inspection, the nodule was 11 mm × 8 mm × 3 mm, smooth surfaced and firm without infiltration into underlying tissue. Histopathology analysis revealed a dense lymphoplasmacytic cell infiltrate with lymphoid follicle formation and abundant eosinophilic infiltration in the background of fibrous stroma. There were proliferating capillaries lined with plump endothelial cells [Figure 1]c, [Figure 1]d, [Figure 1]e. There was no evidence of granuloma, dysplasia, endothelial atypia, or hobnailing. No storiform fibrosis or obliterative phlebitis was noted. The histological features were suggestive of Kimura's disease. On review of her systemic investigations, her serum immunoglobulin E (IgE) and immunoglobulin A levels, urine protein levels, as well as chest X-ray were within the normal limits. She showed a well-healed ocular surface at 6-weeks of follow-up [Figure 2]. She continues to do well at the end of 12-months of follow-up with no signs of local recurrence or lymphadenopathy.
Figure 2: Preoperative and postoperative picture. (a) Preoperative slit-lamp image and (b) postoperative image 6-weeks of follow-up showing healthy ocular surface

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  Discussion Top

KD is documented to occur mainly in Asian males, as large (>2 cm) subcutaneous papules or nodules with accompanying elevated blood eosinophil count (up to 54%), hypergammaglobulinemia (IgE), regional lymphadenopathy, and salivary gland involvement.[6],[7] The clinical and pathologic findings of KD are historically known to overlap with those of angiolymphoid hyperplasia with eosinophilia (ALHE), a benign neoplasm of endothelial cells. The terms KD and ALHE have been used synonymously in many reports and reviews in published literature. Histological examination is said to provide the differentiation between the two. The character of endothelial cells is of particular diagnostic importance as atypical endothelial cells are not seen in KD, while ALHE is defined by the presence of proliferation of the atypical histiocytoid endothelial cells. In KD, lymphoid follicles and abundant fibrous tissue are present (as in the case described). On the other hand, ALHE shows a relative scarcity of lymphoid follicles and fibrosis.[4]

After a detailed review of the English literature with keywords “Kimura's disease,” “conjunctiva” or “ocular surface” we found a case report by Jayamanne et al. describing an ulcerated lesion of the conjunctiva in a 24-year-old girl then diagnosed as ALHE or KD.[8] However, the authors did not comment on the pathologies being a separate entity from each other. Kim et al. reported a case of KD involving the caruncle in 35-year-old female as the first report of caruncular involvement.[9] Esmaili et al. described eyelid mass consistent with KD and a left conjunctival mass consistent with ALHE and compared these two disease entities.[10] We did not find a reported case of Kimura's involving bulbar conjunctiva with its clinicopathological details. Thus, the authors report the first case of KD presenting as a nodule involving bulbar conjunctiva in a 50-year-old female.

Involvement of orbit and ocular adnexa by KD as well as ALHE is rare.[4],[10] Buggage et al. reviewed 33 cases involving the orbit and ocular adnexa and reported orbit as the most affected site.[4] Our patient was a middle-aged female presenting with a solitary bulbar conjunctival nodule and distinguishing histology features without lymphadenopathy or hypergammaglobulinemia (IgE).

The standard treatment for KD in the ocular region is not determined. Complete surgical excision is the most widely used modality, while oral or intralesional steroids, radiation, and chemotherapy can also be utilized.[4] Recurrence of the lesion is common after incomplete excision.[2] Our patient was treated with complete surgical excision and local recurrence was not detected at follow-up of 12-months.

Solitary conjunctival nodule can be a presenting feature of nodular episcleritis or scleritis, foreign body granuloma, lymphoproliferative disease, or occasionally epithelial or stromal tumors.[11] Atypical features, progressive increase in size or suboptimal response to treatment with topical steroids can be considered an indication for biopsy for such lesions. A complete excision biopsy in case of well-defined lesion like this can be both diagnostic as well as therapeutic. The histological features help in achieving correct diagnosis and predicting further prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kimura T, Yoshimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissue. Trans Soc Pathol Jpn 1948;37:179-80.  Back to cited text no. 1
Kung IT, Gibson JB, Bannatyne PM. Kimura's disease: A clinico-pathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology 1984;16:39-44.  Back to cited text no. 2
Kuo TT, Shih LY, Chan HL. Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol 1988;12:843-54.  Back to cited text no. 3
Buggage RR, Spraul CW, Wojno TH, Grossniklaus HE. Kimura disease of the orbit and ocular adnexa. Surv Ophthalmol 1999;44:79-91.  Back to cited text no. 4
Lee SJ, Song JH, Kim SD. Kimura's disease involving the ipsilateral face and extraocular muscles. Korean J Ophthalmol 2009;23:219-23.  Back to cited text no. 5
Googe PB, Harris NL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two distinct histopathological entities. J Cutan Pathol 1987;14:263-71.  Back to cited text no. 6
Urabe A, Tsuneyoshi M, Enjoji M. Epithelioid hemangioma versus Kimura's disease. A comparative clinicopathologic study. Am J Surg Pathol 1987;11:758-66.  Back to cited text no. 7
Jayamanne DG, Webber SK, Ridley RE, Cottrell DG, McCarthy JH. Angiolymphoid hyperplasia with eosinophilia (Kimura's disease) of the conjunctiva. Br J Ophthalmol 1995;79:1053-4.  Back to cited text no. 8
Kim WJ, Chung MJ, You IC. Kimura disease involving a caruncle. Korean J Ophthalmol 2013;27:137-40.  Back to cited text no. 9
Esmaili DD, Chang EL, O'Hearn TM, Smith RE, Rao NA. Simultaneous presentation of Kimura disease and angiolymphoid hyperplasia with eosinophilia. Ophthalmic Plast Reconstr Surg 2008;24:310-1.  Back to cited text no. 10
Honavar SG, Manjandavida FP. Tumors of the ocular surface: A review. Indian J Ophthalmol 2015;63:187-203.  Back to cited text no. 11
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