Cutaneous Noncaseating Granulomas Associated With Nijmegen Breakage Syndrome

Nijmegen breakage syndrome (NBS) is an autosomal recessive disorder characterized by immunodeficiency, predisposition to cancer, growth retardation, and characteristic facies.¹ This rare syndrome shares molecular and clinical similarities with ataxia-telangiectasia (AT), in which cutaneous granulomas have been described.² To our knowledge, cutaneous granulomas have not been reported with NBS. We describe herein a patient with NBS presenting with noncaseating granulomas confined to the skin.