Emergence of solitary juvenile xanthogranuloma on the corneoscleral limbus

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.