Juvenile Xanthogranuloma

INTRODUCTION Juvenile xanthogranuloma (JXG) is a rare systemic childhood disease of non-Langerhans cell histiocytes. It is characterized by cutaneous and, on occasion, intraocular lesions. It may be a gran-ulomatous reaction of histiocytes to an unidentified stimulus. There is special predilection for skin and eye involvement. It affects children below the age of five years with 85% of the cases being under one year of age. Most patients are younger than two years of age at presentation. There is no sexual or racial predilection. Other sites of ocular involvement include the orbit, conjunctiva, cornea, episclera, iris, and ciliary body. Iris lesions are associated with spontaneous hyphema, unilateral glaucoma, uveitis, and heterochromia iridis. Although lesions are common on the face and neck, they may also occur on the trunk or extremities. Visceral involvement is rare, however, with a predilection for lung, spleen, testis, pericardium and gastrointestinal tract.

CLINICAL PRESENTATION The eyelid is the most frequent site of ocular involvement. Lesions on the lid often appear quite suddenly. They typically present as a rapidly growing solitary papular or nodular lesion with a rubbery or cartilaginous consistency, varying in size from few millimeters to 1.5 cm. The lesions are typically yellow in color, frequently with an orange overtone. With time they may enlarge and become yellow-brown. New lesions can arise within a few months of the appearance of the first lesion, near or distant to the primary site. The lesions may resolve spontaneously within months or a few years, leaving a slightly atrophic area that may be hyper or hypopigmented. Very large lesions may show ulceration and crusting. Intraocular lesions can be associated with glaucoma and even blindness.

Langhans Cell Touton
(Courtesy of Morris Hartstein, M.D.) (Courtesy of David Lyon, M.D.)

HISTOPATHOLOGY Lipid-laden macrophages (foam cells) and Touton giant cells are the distinguishing features of xanthogranulomas. The Touton giant cells have multiple nuclei forming a circle near the middle of the cell surrounding a core of brightly eosinophilic cytoplasm. The cytoplasm around the outside of the nuclei is vacuolated and clear or palely eosinophilic. Lymphocytes, plasma cells, and occasional eosinophils are scattered among the foam cells and Touton giant cells.

Differential Diagnosis Eosinophilia

DIFFERENTIAL DIAGNOSIS Xanthomas or xanthelasmas, syringoma, and molluscum contagiosum most commonly mimic eyelid involvement of JXG.

TREATMENT Surgical biopsy may be necessary to establish the diagnosis. Treatment of juvenile xanthogranulo-ma should be tailored to the individual case. The majority of lesions are asymptomatic, and may sometimes resolve spontaneously. For isolated skin lesions limited to the eyelid or the epibulblar tissue, observation alone may be appropriate. Skin lesions may be removed surgically for improved cosmesis. For intraocular lesions subconjunctival or systemic corticosteroid therapy, irradiation, and surgery have been reported to give a good response. With systemic visceral involvement, chemotherapy is indicated.

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Responses

  • Savanna Gardner
    Can juvenile xanthogranulomas be removed?
    6 years ago

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