Atypical Necrobiotic Xanthogranuloma Associated with Epidermodysplasia Verruciformis

Necrobiotic xanthogranuloma is a rare, idiopathic, and chronic systemic granulomatous disorder that is usually associated with paraproteinemia of the immunoglobulin (Ig)G kappa (κ) type. It is clinically characterized by yellowish red-to-brown indurated often ulcerating nodules or plaques that usually involve the periorbital regions of the elderly. We report an atypical case of NXG affecting only the left leg of an adolescent girl with both IgG kappa and Lambda paraproteinemias. In addition, she had associated epidemodysplasia verruciformis and severe lung disease.