Ortiz-Romero P L, Corell-Almuzara A, Lopez-Estebaranz J L, Arranz F R, Ruiz-Contreras J
Service of Dermatology, Hospital 12 de Octubre, Universidad Complutense, Madrid, Spain.
Dermatology. 1997;195(3):280-3. doi: 10.1159/000245963.
Lupus-like lesions in X-linked chronic granulomatous disease (X-CGD) are rare. To our knowledge, only 2 cases have previously been published. We report a 2.5-year-old boy with X-CGD whose clinical findings were consistent with cutaneous lupus erythematosus. Conventional histopathology showed epidermal atrophy, parakeratosis, follicular plugging and areas of hydropic degeneration. The most striking feature was a neutrophilic interstitial infiltrate with leukocytoclasia in the upper dermis. The X chromosome of our patient--studied with 2 endonucleases (PstI and TaqI) and 5 probes (P99.6, pERT 87.8, pERT87.15, XJ1.1 and 754)--was recombinant, but we believe that this is an incidental finding, not related to the disease. Neutrophilic infiltrate and leukocytoclasia could be characteristic histopathologic findings of lupus-like lesions in these patients.
X连锁慢性肉芽肿病（X-CGD）中的狼疮样病变较为罕见。据我们所知，此前仅发表过2例。我们报告了一名2.5岁患X-CGD的男孩，其临床 findings 与皮肤红斑狼疮一致。传统组织病理学显示表皮萎缩、角化不全、毛囊堵塞及水肿变性区域。最显著的特征是真皮上层有中性粒细胞间质浸润伴核尘。我们的患者的X染色体——用2种核酸内切酶（PstI和TaqI）及5种探针（P99.6、pERT 87.8、pERT87.15、XJ1.1和754）进行研究——是重组的，但我们认为这是一个偶然发现，与疾病无关。中性粒细胞浸润和核尘可能是这些患者狼疮样病变的特征性组织病理学 findings。
