Abstract

Atypical Necrobiotic Xanthogranuloma Associated with Epidermodysplasia Verruciformis

Highlights

  • First described by Kossard and Winkelmann in 1980 [1], Necrobiotic xanthogranuloma (NXG) is a rare, chronic, idiopathic, progressive, locally destructive, systemic histiocytic disorder affecting primarily the skin

  • We report an atypical case of NXG affecting only the left leg of an adolescent girl with both IgG kappa and Lambda paraproteinemias

  • The extremities can be affected without periorbital involvement [4,5,6,7]

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Summary

Introduction

First described by Kossard and Winkelmann in 1980 [1], Necrobiotic xanthogranuloma (NXG) is a rare, chronic, idiopathic, progressive, locally destructive, systemic histiocytic disorder affecting primarily the skin. We report an atypical case of NXG affecting only the left leg of an adolescent girl with both IgG kappa and Lambda paraproteinemias. The patient had associated epidemodysplasia verruciformis (EDV) and severe lung disease.

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