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Year : 2018  |  Volume : 6  |  Issue : 2  |  Page : 74-76

Emergence of solitary juvenile xanthogranuloma on the corneoscleral limbus

1 Tecnologico de Monterrey, School of Medicine and Health Sciences, Ophthalmology Residency Program, Monterrey, Mexico
2 Centro Medico Zambrano Hellion, Institute of Ophthalmology and Visual Sciences, San Pedro Garza García, Mexico

Date of Web Publication20-Jun-2018

Correspondence Address:
Alejandro Rodriguez-Garcia
Instituto de Oftalmología y Ciencias Visuales, Centro Médico Zambrano Hellion, Av. Batallón de San Patricio No. 112, Col. Real de San Agustín, San Pedro Garza García, Nuevo León, CP. 66278
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcor.jcor_11_17

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Juvenile xanthogranuloma (JXG) is the most frequent form of non-Langerhans histiocytosis, a rare benign inflammatory skin disorder. Eye involvement is the most frequent extracutaneous manifestation of the disease. Limbal JXG is an uncommon ocular presentation. We present a 7-year-old White female child who developed a limbal nodule suspicious of JXG with no cutaneous manifestations. Due to rapid progression and no response to topical steroids, the lesion was surgically excised. Histopathological analysis revealed large numbers of foamy histiocytes, Touton multinucleated giant cells, and lymphocytic infiltration. Immunohistochemical staining showed cellular expression of the histiocytic marker: CD68, and absent expression of Langerhans cell marker: CD1a. A definitive diagnosis must be made in such cases to rule out other limbal lesions presenting during childhood such as dermoid, dermatofibroma, neurofibroma, and eosinophilic granuloma. In addition, because spontaneous regression of the lesion is improbable, surgical excision is recommended to avoid its recurrence and ocular complications.

Keywords: Corneoscleral limbus, histiocytosis, non-Langerhans, Touton cells, xanthogranuloma

How to cite this article:
Alfaro-Rangel R, Paez-Garza J H, Rodriguez-Garcia A. Emergence of solitary juvenile xanthogranuloma on the corneoscleral limbus. J Clin Ophthalmol Res 2018;6:74-6

How to cite this URL:
Alfaro-Rangel R, Paez-Garza J H, Rodriguez-Garcia A. Emergence of solitary juvenile xanthogranuloma on the corneoscleral limbus. J Clin Ophthalmol Res [serial online] 2018 [cited 2023 Jun 8];6:74-6. Available from: https://www.jcor.in/text.asp?2018/6/2/74/234818

Juvenile xanthogranuloma (JXG) is a rare, benign, and inflammatory skin disorder of unknown etiology that occurs at pediatric age.[1] It is the most frequent form of non-Langerhans cell histiocytosis.[2] Cutaneous lesions generally appear early in life as small reddish-yellow macules, papules, or nodules in the head and neck, which resolve spontaneously without any treatment.[1]

The most frequent extra-cutaneous manifestation of JXG occurs in the eye, but lesions may also appear in the brain, lungs, liver, spleen, and other sites.[1] Eye involvement is infrequent, appearing in only 0.3%–10% of children with cutaneous JXG.[3] Ocular lesions usually consist of a solitary iris granuloma, but lesions on the cornea, conjunctiva, eyelids, choroid, optic nerve, and orbit have also been described.[4] The iris, conjunctiva, and eyelids are the most frequently involved ocular structures of JXG.[5],[6] Limbal lesions affecting the conjunctiva, episclera, and cornea are a rare manifestation of JXG.[7] The purpose of this report is to discuss the differential diagnosis and management of limbal lesions appearing during childhood.

  Case Report Top

A 7-year-old White female child was referred to our service for evaluation of a conjunctival mass in the right eye noticed 3 weeks before consultation by her mother. The lesion appeared suddenly and grew rapidly as a yellow-orange, round conjunctival bump adjacent to the right cornea. The patient's medical history, as well as the general physical examination, was unremarkable. On ocular examination, a raised, fixed yellow-orange, vascularized, and well-delimitated lesion of 2–3 mm in diameter was observed in the superotemporal limbal conjunctiva. After finger depression through the eyelid, the lesion collapsed and whitened for a few seconds, returning to its original aspect as blood vessels gained recirculation [Figure 1]. Under slit-lamp examination, no eyelid, iris, or any other intraocular lesion was noted. Visual acuity was 20/20 in both eyes and the rest of the ocular examination was normal. The skin was not involved, and on direct interrogation to her mother, she had not noticed any previous cutaneous lesions over her entire body. The patient was initially managed with 1% fluorometholone acetate three times a day, but after 10 days on therapy, the lesion had enlarged, measuring 5 mm in diameter, became firm, and had a reddish nodular aspect [Figure 2]. After discussion with her parents, it was decided to remove the lesion. En bloc excision biopsy was planned under general anesthesia. The lesion was dissected from the firmly attached episclera at a 2-mm larger demarcation area, removed in one piece, and sent for histopathologic examination which showed abundant foamy histiocytes, lymphocytic inflammatory reaction, and Touton multinucleated giant cells [Figure 3]. Immunohistochemical staining showed a marked expression of the histiocytic marker CD68 but no expression of the CD1a Langerhans cell marker [Figure 4]. The cells did not express the metastatic malignant melanoma markers, S-100 protein, HMB-45, and cytokeratin AE1/AE3. Based on the clinical aspect of the lesion and the histopathologic findings, a definitive diagnosis of JXG of limbal conjunctiva was made. The patient was followed up closely over a 3-year period, showing no evidence of recurrence of the limbal lesion, or the appearance of any skin manifestation.
Figure 1: Superotemporal limbal conjunctiva lesion at presentation. (a) Note the pale, translucid yellow-orange color. (b) After depression, the lesion collapsed and whitened for a few seconds before returning to its original aspect

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Figure 2: Ten days after topical steroid therapy, the lesion enlarged, looked denser, and had an orange-reddish nodular aspect

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Figure 3: Histopathologic micrograph of the lesion showing abundant histiocytic and lymphocytic infiltration of the conjunctival stroma, accompanied by Touton multinucleated giant cells (H and E, ×10)

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Figure 4: Immunohistochemical analysis of the c onjunctival lesion showing strong expression of the histiocyte marker CD68 (IHC-P, ×10)

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  Discussion Top

Solitary JXG affecting the corneoscleral limbus is a rare form of ocular manifestation of the disease, with only 21 cases reported in the literature so far.[5],[7] The majority of reported cases are unilateral as ours. Interestingly, there is a low prevalence of skin manifestations in patients with ocular JXG and particularly in the limbal presentation (only 4/21).[5],[6],[7] In the present case, no skin lesions have appeared after 3 years of follow-up.

Histologically, all limbal lesions have demonstrated typical findings of JXG, with large numbers of foamy histiocytes, Touton multinucleated giant cells, and inflammatory infiltration consisting predominantly of lymphocytes and eosinophils.[8] Xanthogranulomatous lesions express the macrophage–histiocytic markers, CD68, HAM56, cathepsin-B, and vimentin, and must be differentiated from Langerhans cell histiocytic lesions where cells express CD1a and langerin.[8] Our patient's immunohistochemical profile proved a non-Langerhans lesion with absent expression for melanoma markers. A similar case of corneal XG has also been reported in an adult.[9]

The differential diagnosis of a mass lesion occurring in the corneoscleral limbus of an infant includes dermoid, mastocytoma, dermatofibroma, neurofibroma, amelanotic melanoma, Spitz nevi, foreign body granuloma, eosinophilic granuloma, and phlyctenular keratoconjunctivitis, among other inflammatory lesions.[1],[3] The great majority of these disorders may be excluded from clinical history and appearance of the lesion. The final diagnosis, however, depends on excisional biopsy when the diagnosis is uncertain, particularly in cases such as neurofibromas which must be distinguished from neuromas that may be associated with multiple endocrine neoplasia.[8],[10] The same holds true for conjunctival melanoma which can be mistaken with a Spitz nevus, a benign melanocytic lesion of mucosal origin that may arise in the limbal conjunctiva.[8],[10]

When the diagnosis of JXG is made, skin lesions should be searched for; however, their absence does not rule out JXG since cutaneous lesions may appear later, and particularly in most cases of limbal JXG, they are absent.[1],[5],[6] Another rare systemic association of XG with MEN1 has been reported but not in the pediatric age group.[9]

The mechanism of how JXG lesions appear is not clear, but the macrophage differentiation observed in the histiocytes of these lesions suggests an inflammatory reaction to an unknown stimulus.[8],[11] Topical and systemic steroids, radiotherapy, methotrexate, and recently anti-vascular endothelial growth factor agents have been used with variable results; however, since spontaneous regression of limbal lesions is unlikely, excision of the lesion is recommended to avoid complications.[9],[10],[11]

  Conclusion Top

Solitary JXG presenting as a rapidly growing corneoscleral limbus nodule is an unusual ocular manifestation of the disease. As with iris JXG granulomas, a definitive diagnosis and therapy consisting of surgical excision of the entire lesion are recommended.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Dehner LP. Juvenile xanthogranulomas in the first two decades of life: A clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 2003;27:579-93.  Back to cited text no. 1
Margo CE, Goldman DR. Langerhans cell histiocytosis. Surv Ophthalmol 2008;53:332-58.  Back to cited text no. 2
Liang S, Liu YH, Fang K. Juvenile xanthogranuloma with ocular involvement. Pediatr Dermatol 2009;26:232-4.  Back to cited text no. 3
Longmuir S, Dumitrescu A, Kwon Y, Boldt HC, Hong S. Juvenile xanthogranulomatosis with bilateral and multifocal ocular lesions of the iris, cornealscleral limbus, and choroid. J AAPOS 2011;15:598-600.  Back to cited text no. 4
Zimmerman LE. Ocular lesions of juvenile xanthogranuloma. Nevoxanthoedothelioma. Am J Ophthalmol 1965;60:1011-35.  Back to cited text no. 5
Samara WA, Khoo CT, Say EA, Saktanasate J, Eagle RC Jr., Shields JA, et al. Juvenile xanthogranuloma involving the eye and ocular adnexa: Tumor control, visual outcomes, and globe salvage in 30 patients. Ophthalmology 2015;122:2130-8.  Back to cited text no. 6
Chaudhry IA, Al-Jishi Z, Shamsi FA, Riley F. Juvenile xanthogranuloma of the corneoscleral limbus: Case report and review of the literature. Surv Ophthalmol 2004;49:608-14.  Back to cited text no. 7
Sangüeza OP, Salmon JK, White CR Jr., Beckstead JH. Juvenile xanthogranuloma: A clinical, histopathologic and immunohistochemical study. J Cutan Pathol 1995;22:327-35.  Back to cited text no. 8
Alkatan HM, Al-Rajhi AA. Corneal xanthogranuloma in association with multiple endocrine neoplasia 1: A clinicopathologic case report and review of the literature. Saudi J Ophthalmol 2016;30:49-52.  Back to cited text no. 9
Mocan MC, Bozkurt B, Orhan D, Kuzey G, Irkec M. Juvenile xanthogranuloma of the corneal limbus: Report of two cases and review of the literature. Cornea 2008;27:739-42.  Back to cited text no. 10
Kontos G, Borooah S, Khan A, Fleck BW, Coupland SE. The epidemiology, clinical characteristics, histopathology and management of juvenile- and adult-onset corneoscleral limbus xanthogranuloma. Graefes Arch Clin Exp Ophthalmol 2016;254:413-20.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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