Spectacular skin nodules: cutaneous necrobiotic xanthogranuloma without paraproteinemia.

Necrobiotic xanthogranuloma (NXG) is a very rare, progressive variant of non-Langerhans cell histiocytosis. It is known to be associated with multisystem involvement and paraproteinemias. A 65-year-old female presented with chronic, slowly growing, asymptomatic periorbital nodules. The lesions had recurred after local excision elsewhere. No systemic involvement or paraproteinemias were detected. A provisional diagnosis of isolated cutaneous NXG was made which was confirmed by histopathology and immunohistochemistry staining. The lesions were surgically excised with excellent cosmetic and functional results. There was no recurrence over a period of 9 months. To our knowledge, this is the second case of NXG reported from India and the first without any systemic manifestations.