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Arch Ophthalmol. 2003;121:117-118.

Juvenile xanthogranuloma (JXG) is a benign histiocytic disease of uncertain pathogenesis that usually appears as a cutaneous lesion(s) in the head and neck region of young children. Diagnosis is based on histological examination that shows a nodular, dense infiltrate of polygonal or spindled mononuclear cells with moderate amounts of cytoplasm and scattered Touton giant cells. Juvenile xanthogranulomas are typically positive for CD68 and factor XIIIa immunostains and are negative for CD1a and S100 protein; this information is helpful in differentiating JXG from other histiocytic proliferations.¹ We present the case of a 4-month-old child with corneal JXG.

Report of a Case
The mother of a 4-month-old infant boy noted an enlarging, raised mass in the medial corner of his right eye. A biopsy performed by a local ophthalmologist was interpreted as noncaseating granulomatous inflammation. Despite normal findings on systemic evaluation, sarcoidosis was diagnosed. He received topical steroid therapy and was referred to us.

Examination of the patient's right eye at 11 months revealed an elevated 12 x 8-mm yellow epibulbar mass involving the temporal conjunctiva, overlying the medial rectus muscle, extending onto the cornea to the nasal pupillary margin, and invading the corneal stroma (Figure 1). No prominent blood supply was present. His vision was centered, steadied, and maintained. Cycloplegic refraction was plano +2.50 x 90 OD and +0.75 sphere OS. A dilated fundus examination was normal. No refractive error was present. A review of original biopsy material showed a JXG, with a dense nodular infiltrate beneath the corneal epithelium comprising polygonal cells with moderate amounts of eosinophilic cytoplasm and rare Touton giant cells (Figure 2). Immunohistochemical studies showed strong positivity for vimentin and CD68, weak staining for factor XIIIa, and negative staining for S100 protein and CD1a. No evidence of necrosis was noted. The patient was treated with a topical steroid regimen of loteprednol etabonate every 2 hours, and fluorometholone ointment nightly with no complications. Follow-up examinations showed regression of the mass during 12 months, with associated scarring of the cornea.

View larger version (139K): [in this window] [in a new window] Figure 1. Right eye of an 11-month-old child. Note the yellow corneoscleral limbus nodule with prominent vasculature and lipid deposition in the corneal stroma.

View larger version (201K): [in this window] [in a new window] Figure 2. Corneal juvenile xanthogranuloma with dense nodular infiltrate beneath the epithelium, comprising polygonal cells with moderate amounts of eosinophilic cytoplasm intermixed with chronic inflammatory cells. Rare Touton giant cells are seen (inset) (hematoxylin and eosin, original magnification x100 [inset, original magnification x400]).

Comment
A benign proliferation of cells of the monocyte/macrophage or dendritic cell lineage, JXG represents one of several "non-Langerhans cell"–type histiocytoses. In 80% of cases, JXG appears in the first 6 to 9 months of life as solitary or multiple red or brown cutaneous nodules.¹ The lesions most commonly occur in the head and neck region, the upper part of the trunk, or the proximal limbs. The natural history is usually one of spontaneous involution after several months.

Ocular involvement occurs in 10% of cases of JXG.² The iris is the most common location, followed by the eyelid, choroid, optic nerve, retina, conjunctiva, limbus, and cornea.² Thirteen cases involving the corneoscleral limbus are documented, 6 exhibiting extension onto the cornea in patients aged 18 months through 30 years.^3-5 Although topical steroid therapy has proven successful in managing lesions of the iris, its usefulness for JXG involving the cornea has not been established.² Of the 6 cases with corneal involvement, 4 were treated with excision and graft placement, one with excision followed by irradiation, and the last with simple excision.^3-5 In deciding on a treatment strategy, we weighed the risks of topical steroids against those of surgery. Because this lesion did not produce a significant refractive error, we elected to pursue a conservative course of therapy with topical steroids. At 12 months' follow-up, the lesion seemed to be regressing, although a corneal scar remains.

Emily D. Ashmore, MD; Matthew W. Wilson, MD, FACS; William R. Morris, MD; D. Ashley Hill, MD; Carlos Rodriguez-Galindo, MD; Barrett G. Haik, MD, FACS
Memphis, Tenn

Corresponding author and reprints: Matthew W. Wilson, MD, Department of Ophthalmology, University of Tennessee Health Science Center, 956 Court Ave, D-228, Memphis, TN 38163 (e-mail: mwilson{at}mail.eye.utmem.edu).

REFERENCES
1. Dehner LP. Juvenile xanthogranuloma (JXG) in children: a clinicopathologic study of 179 cutaneous and extracutaneous cases [abstract]. Mod Pathol. 2002;15:2P. 2. Zimmerman LE. Ocular lesions of juvenile xanthogranuloma. Am J Ophthalmol. 1965;60:1011-1035. ISI | PUBMED 3. Yanoff M, Perry HD. Juvenile xanthogranuloma of the corneoscleral limbus. Arch Ophthalmol. 1995;113:915-917. ABSTRACT 4. Harvey P, Lee JA, Talbot JF, Goepel JR. Isolated xanthogranuloma of the limbus in an adult. Br J Ophthalmol. 1994;78:657-659. FREE FULL TEXT 5. Lewis JR, Drummond GT, Mielke BW, Hassard DT, Astle WF. Juvenile xanthogranuloma of the corneoscleral limbus. Can J Ophthalmol. 1990;25:351-354. ISI | PUBMED

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