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March> 1, 2008

Cutaneous Noncaseating Granulomas Associated With Nijmegen Breakage Syndrome

Arch Dermatol. 2008;144(3):418-419. doi:10.1001/archderm.144.3.418

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.

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