Shoimer Ilya, Wright Nicola, Haber Richard M
Division of Dermatology, University of Calgary, Calgary, AB, Canada.
Division of Pediatric Hematology/Immunology, University of Calgary, Calgary, AB, Canada.
J Cutan Med Surg. 2016 May;20(3):259-62. doi: 10.1177/1203475415626085. Epub 2016 Jan 4.
Primary immunodeficiency disorders, such as ataxia-telangiectasia (A-T), may rarely be associated with cutaneous granulomas without an identifiable infection. The authors report a case of a 3-year-old boy with A-T who presented with two persistent ulcerated erythematous nodules. Histopathology was consistent with a granulomatous process secondary to A-T, without an infectious origin. Partial improvement was noted with clobetasol propionate 0.05% cream applied twice daily under occlusion. Of note, the presence of multiple noninfectious granulomas in a child may be the initial sign of an immune deficiency and should alert the astute clinician to investigate for an underlying primary immunodeficiency. Herein, the authors discuss the associations of noninfectious granulomas and primary immunodeficiency disorders and present management options for these difficult-to-treat lesions.
原发性免疫缺陷病，如共济失调毛细血管扩张症（A-T），极少情况下可能与无明确感染的皮肤肉芽肿相关。作者报告了一例3岁患A-T的男孩，其出现了两个持续存在的溃疡红斑结节。组织病理学与A-T继发的肉芽肿过程一致，无感染源。每天两次在封包下外用0.05%丙酸氯倍他索乳膏后可见部分改善。值得注意的是，儿童出现多个非感染性肉芽肿可能是免疫缺陷的初始迹象，应提醒敏锐的临床医生调查潜在的原发性免疫缺陷。在此，作者讨论了非感染性肉芽肿与原发性免疫缺陷病的关联，并提出了针对这些难以治疗的皮损的管理方案。
