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BRIEF COMMUNICATION
Year : 2015  |  Volume : 3  |  Issue : 1  |  Page : 27-28

Intraocular juvenile xanthogranuloma masquerading as conjunctivitis in an adult


1 Department of Ophthalmology, Ocular Oncology, Oculoplasty Service; Axis Eye Clinic, Pune, Maharashtra, India
2 Department of Ophthalmology, Uveitis Service, Pune, Maharashtra, India
3 Department of Pathology, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India
4 Head of Department, Ophthalmology, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India

Date of Submission12-Jan-2014
Date of Acceptance15-Apr-2014
Date of Web Publication14-Jan-2015

Correspondence Address:
Ramesh Murthy
Department of Ophthalmology, Deenanath Mangeshkar Hospital, Erandawane, Pune - 411004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-3897.149359

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  Abstract 

A 22-year-old female presented to us with redness, pain and intermittent blurring of vision in the left eye of one month duration. She had been diagnosed as a case of conjunctivitis elsewhere for which she was being treated with topical antibiotics. Examination of the anterior segment revealed diffuse conjunctival congestion, the presence of an inferonasal iris bulge and a streak of hyphema. Ultrasound biomicroscopy revealed an irregular echogenic mass lesion in the iris extending into the ciliary body. Fine needle aspiration cytology revealed the presence of large giant cells with multiple nuclei and pigments in the cytoplasm suggestive of Touton giant cells and multiple histiocytes confirming the diagnosis of juvenile xanthogranuloma. The patient was prescribed oral and topical steroids and the lesion resolved. Juvenile xanthogranuloma can masquerade as conjunctivitis and simple aspiration cytology can be helpful in establishing the diagnosis instead of a more invasive incision or excision biopsy.

Keywords: Conjunctivitis, fine needle aspiration cytology, juvenile xanthogranuloma, touton giant cell


How to cite this article:
Murthy R, Palsule A, Joshi S, Bhatt M. Intraocular juvenile xanthogranuloma masquerading as conjunctivitis in an adult. J Clin Ophthalmol Res 2015;3:27-8

How to cite this URL:
Murthy R, Palsule A, Joshi S, Bhatt M. Intraocular juvenile xanthogranuloma masquerading as conjunctivitis in an adult. J Clin Ophthalmol Res [serial online] 2015 [cited 2022 May 25];3:27-8. Available from: https://www.jcor.in/text.asp?2015/3/1/27/149359

Juvenile xanthogranuloma (JXG) is a rare, benign cutaneous disorder, which occasionally involves the eye, orbit and ocular adnexa in children. [1] Presentation usually occurs in childhood with spontaneous regression by adolescence. [1] Ocular manifestations usually involve the iris, sometimes leading to spontaneous hyphema but also the eyelids and skin. [2] We describe a rare case of an adult female who presented with an iris mass and was being mistakenly treated as conjunctivitis. Diagnosis was established by simple fine needle aspiration cytology from the mass.


  Case Report Top


A 22-year-old female presented with redness, pain and intermittent blurring of vision in the left eye for a month. She had been diagnosed as a case of conjunctivitis elsewhere, for which she was being treated with topical antibiotics. There was no history of trauma or any antecedent illness. Visual acuity was 6/6 in the right eye and 6/9 in the left eye for distance and near vision was N6 in the right eye and N8 in the left eye. The left eye showed diffuse conjunctival congestion, predominantly in the inferior quadrant. Anterior chamber had a streak of hyphema, the iris had a muddy appearance with fluffy exudates and an inferonasal iris bulge from the 6'oclock to the 8 o'clock position involving the peripheral 2 mm of the iris [Figure 1]. Posterior synechiae was seen at 7 o'clock position. The pupil was sluggishly reacting to light and not dilating with mydriatics or cycloplegics. Fundus examination was within normal limits in both eyes. The intraocular pressure by applanation tonometry was 12 mmHg in the right eye and 14 mmHg in the left eye. Ultrasound biomicroscopy revealed marked thickening of the iris inferiorly with a large irregular echogenic mass lesion (2.2 × 1.1 × 0.5 mm) inferiorly in the iris stroma extending into the ciliary body [Figure 2]. The ciliary body and the ciliary processes were thickened inferiorly. X-ray of the orbit did not reveal any foreign body. Anterior chamber tap and fine needle aspiration cytology of the mass was performed to establish the diagnosis. Cytological examination revealed the presence of large giant cells with multiple nuclei and pigment in the cytoplasm suggestive of Touton giant cells [Figure 3]a with abundant histiocytes [Figure 3]b suggestive of JXG. A final diagnosis of JXG without any systemic involvement was made. She was prescribed oral prednisolone 40 mg daily, which was tapered weekly and topical prednisolone acetate 1% drops four times a day and homatropine 1% drops two times a day in the left eye. The lesion disappeared completely in 3 weeks. Vision improved to 6/6 and the iris became flat. At last, follow-up of eighteen months after the conclusion of treatment, there was no sign of recurrence.
Figure 1: Left eye showed the presence of a bulge in the inferonasal quadrant from the 6 o'clock to the 8 o'clock position with the presence of hyphema. The pupil was oval and the iris had a flat appearance

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Figure 2: Ultrasound biomicroscopy showed the presence of marked iris thickening inferiorly along with thickening of the ciliary body and ciliary processes

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Figure 3: (a) Histopathology revealed the presence of large multinucleated giant cell with pigments in the cytoplasm suggestive of Touton giant cell and (b) the presence of numerous histiocytes

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  Discussion Top


Juvenile xanthogranuloma is a benign cutaneous disorder, which occasionally involves the eye, orbit and ocular adnexa in children. [3] The iris and ciliary body are commonly involved; retinal and choroidal lesions are very rare. [4] Histologically, JXG is characterized by infiltration of foamy histiocytes, Touton and other multinucleated giant cells and various other inflammatory cells.

Juvenile xanthogranuloma most commonly affects children less than one year of age and nearly 64% are younger than 7 months. [1] Skin lesions are common in this condition. [2] Our patient was an adult and did not manifest any cutaneous lesions. JXG has been reported to occur in adults with the oldest being a 77-year-old patient. [5],[6] The clinical features are more atypical in an older patient. [5] Skin lesions may be absent and the condition may mimic other lesions like a melanoma. [6] Our patient was mistakenly treated as conjunctivitis, as the iris lesion was very subtle. When the hyphema appeared, the diagnosis of foreign body, tuberculous granuloma and other conditions like JXG was considered.

As it is an intraocular lesion, establishing the diagnosis is not easy. Some reports suggest that incision or excision biopsy should be performed as the flocculent debris cannot be easily dislodged for cytopathology. [7] We performed a paracentesis and fine needle aspiration cytology of the mass. The presence of characteristic Touton giant cells leads us to the diagnosis. Immunohistochemical techniques have characterized the histiocytic component of these lesions. The histiocytes are of monocyte macrophage origin or dendritic cells. The usefulness of S-100 as a marker for this tumor is debatable. Some believe that JXG does not stain with anti S-100 antibody unlike the Langerhan's cell histocytosis; however, others have reported that 1-10% of the histiocytes in cutaneous lesions of JXG were dendritic S-100 positive cells. [7] However, immunoreactivity to macrophage-related antibodies like HAM 56 and CD 68 may be present. [2] They suggested that these cells were a component of the tumor and functioned in processing antigen at the peripheral-expanding zones. [8]

JXG can masquerade as many conditions. Atypical presentations are more common in adults. Fine needle aspiration cytology through a paracentesis can be useful in establishing the diagnosis. Most lesions respond to steroids, though some may need radiation and other adjunctive therapy. [3] Left untreated they can have recurrent hyphema and intractable glaucoma leading to loss of sight.

 
  References Top

1.
Zimmerman LE. Ocular lesions of juvenile xanthogranuloma. Nevoxanthoendothelioma. Am J Ophthalmol 1965;60:1011-35.  Back to cited text no. 1
[PUBMED]    
2.
Hernandez-Martin A, Baselga E, Drolet BA, Easterly NB. Juvenile xanthogranuloma. J Am Acad Dermatol 1997;36:355-67.  Back to cited text no. 2
    
3.
MacLeod PM. Juvenile xanthogranuloma of the iris managed with superficial radiotherapy. Clin Radiol 1986;37:295-6.  Back to cited text no. 3
[PUBMED]    
4.
Wertz FD, Zimmermann LE, McKeown CA, Croxatto JO, Whitmore PV, LaPiana FG. Juvenile xanthogranuloma of the optic nerve, disc, retina and choroid. Ophthalmology 1989;89:1331-5.  Back to cited text no. 4
    
5.
Smith ME, Sanders TE, Bresnick GE. Juvenile xanthogranuloma of the ciliary body in an adult. Arch Ophthalmol 1969;81:813-4.  Back to cited text no. 5
    
6.
Sukavatcharin S, Cursino S, Li G, Green RL, Lim JI, Rao NA. Xanthogranuloma of the iris simulating melanoma in an adult. Am J Ophthalmol 2007;143:529-31.  Back to cited text no. 6
    
7.
Fontanilla FA, Edward DP, Wong M, Tessler HH, Eagle RC, Goldstein DA. Juvenile xanthogranuloma masquerading as melanoma. J AAPOS 2009;13:515-8.  Back to cited text no. 7
    
8.
Busam KJ, Rosai J, Iversen K, Jungbluth AA. Xanthogranulomas with inconspicuous foam cells and giant cells mimicking malignant melanoma: A clinical, histologic and immunohistochemical study of three cases. Am J SurgPathol 2000;24:864-9.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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2 New Ultrasound Biomicroscopy Iris Findings in Juvenile Xanthogranuloma
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