Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement

To report long-term outcomes of patients with necrobiotic xanthogranuloma, to investigate the propriety of therapeutic surgical excision or debulking, and to study tissue specimens by immunoperoxidase staining and in situ hybridization.

Medical records of all patients at the Mayo Clinic, Rochester, Minnesota, with necrobiotic xanthogranuloma between 1980 and 1997 were reviewed. A follow-up letter was sent to each patient inquiring about the current status of the lesions, the treatment regimen, and associated systemic diseases.

The average age (+/- standard deviation) of the 15 men and 11 women was 56.8 +/- 14.8 years. Of the 26 patients, 21 (81%) had lesions of the ocular adnexa. Ulceration of the lesions occurred in 11 patients (42%). The lesions recurred after surgical removal in 11 patients (42%) and on prior incision sites from unrelated operations in three patients (12%). The average duration of follow-up from the appearance of characteristic skin lesions was 10 +/- 6.1 years. Four patients had multiple myeloma, five had a plasma cell dyscrasia, and one had a lymphoproliferative disorder during this period. Time to development of associated malignancy ranged from 8 years before the skin lesions to 11 years after the skin lesions. Overall survival was 100% at 10 years and 90% at 15 years (95% confidence limit, 0.73 to 1.00). Immunoperoxidase stains demonstrated that most histiocytes are not of Langerhans cell lineage. Monoclonal immune globulins were not identified in tissue specimens.

Care of patients with necrobiotic xanthogranuloma should include avoidance of surgical removal, if possible, and lifelong follow-up to detect the development of associated malignancy.