X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disease. We reported two 7-month-old identical male twins with Pseudomonas aeruginosa sepsis who initially manifested as oral ecthyma gangrenosum and were finally diagnosed to have XLA. In both cases, we confirmed the c.862C > T BTK missense mutation in exon 10 at the SH2 domain.

中文翻译：

---

铜绿假单胞菌败血症在布鲁顿酪氨酸激酶基因突变的同卵双胞胎中表现为口腔坏疽性腐烂：两例病例并文献复习。

X连锁无球蛋白血症（XLA）是一种主要的免疫缺陷疾病。我们报道了两个7个月大的铜绿假单胞菌败血症的同卵双胞胎，最初表现为口腔坏疽，最后被诊断患有XLA。在这两种情况下，我们都证实了SH2域外显子10中的c.862C> T BTK错义突变。