DeWitt Christine A, Bishop Amanda B, Buescher Lucinda S, Stone Stephen P
Division of Dermatology, Southern Illinois University School of Medicine, Springfield, Illinois, USA.
J Am Acad Dermatol. 2006 May;54(5):855-65. doi: 10.1016/j.jaad.2005.10.022.
Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency associated with elevated serum IgE levels, eczematous skin, recurrent cutaneous infections, and distinctive musculoskeletal features. We report two cases seen at our institution and review the current literature. Patient 1 was an 18-month-old African American boy with recurrent staphylococcal cold abscesses, pneumonia, and bacteremia. He had severely eczematous skin, ultimately complicated by eczema herpeticum. After treatment of systemic infections with culture-directed antibiotics, a brief course of cyclosporine, 5 mg/kg, improved the dermatitis and allowed transition to long-term therapy with oral trimethoprim-sulfamethoxazole. Patient 2 was a 15-year-old Caucasian boy with long-standing HIES. He has been maintained on a regimen of interferon gamma injections given 3 times weekly and monthly intravenous immunoglobulin since the age of 3 years, prophylactic antibiotics, and low-dose fluconazole. He has occasional episodes of cold abscesses and sinusitis, but has had excellent control since institution of this regimen and has not experienced any adverse effects.
高免疫球蛋白E综合征（HIES）是一种罕见的免疫缺陷病，与血清IgE水平升高、湿疹样皮肤、复发性皮肤感染以及独特的肌肉骨骼特征相关。我们报告了在本机构诊治的两例病例，并对当前文献进行综述。病例1是一名18个月大的非裔美国男孩，患有复发性葡萄球菌性冷脓肿、肺炎和菌血症。他有严重的湿疹样皮肤，最终并发疱疹样湿疹。在用针对培养结果的抗生素治疗全身感染后，短期使用5mg/kg的环孢素改善了皮炎，并使其能够过渡到口服甲氧苄啶-磺胺甲恶唑进行长期治疗。病例2是一名15岁的白种男孩，患有长期的HIES。自3岁起，他一直接受每周3次的干扰素γ注射和每月1次的静脉注射免疫球蛋白治疗方案，同时服用预防性抗生素和低剂量氟康唑。他偶尔会出现冷脓肿和鼻窦炎发作，但自采用该治疗方案以来病情得到了很好的控制，且未出现任何不良反应。
