Juvenile xanthogranuloma of the limbus in an adult.

Report of a Case. A 39-year-old man came to our department with a painless limbal mass on his right eye that had enlarged during 3 months. His visual acuity was 20/40 OU and on slitlamp examination, a yellowish, well-circumscribed, vascularized, round nodule was evident at the 6-o’clock position of the right limbus, measuring 6 mm in diameter. The visual axis was clear, and on gonioscopy, neither the trabecular meshwork nor the iris was involved. Further ocular and orbital examination results were unremarkable. Systemic examination revealed an orange-red maculopapular rash involving the trunk, axillae, groin, and face (Figure 1). No associated lymphadenopathy, joint swelling, or oral ulceration were present. Examination of cardiorespiratory and abdominal systems disclosed normal results. Complete blood cell count with differential white cell count, serum lipid and urea levels, creatinine estimation, plasma viscosity, liver function test results, and chest xray film were all normal. Sarcoidosis and tuberculosis were excluded on examination by a pulmonologist. Before a diagnosis could be made, we performed biopsies of the ocular and skin lesions. The histopathologic report of the limbal lesion showed early keratinization over a disorganized spindle cell lesion with scattered lymphocytes and plasma cells. Occasional giant cells were also noted. Histopathologic examination of the skin lesion identified a well-circumscribed lesion composed of histiocyticlooking cells with abundant, occasionally vacuolated cytoplasm, spindle-shaped cells, foci of lymphocytes, and bands of collagen. Multinucleated cells were also found and these occasionally showed the characteristic features of a Touton giant cell. Based on the histopathologic findings, a diagnosis of adult-onset JXG was made. We initially opted for conservative management since the patient was reluctant to undergo surgery. However, 18 months following the initial examination, the lesion had grown to 9 mm 5.7 mm (Figure 2) and was causing some discomfort. Surgical excision was carried out and the lesion was sent for histologic examination. This showed a granulomatous lesion rich in Touton-type giant cells, with occasional Figure 1. Photograph showing typical orange-red maculopapular skin rash.