Wang Audrey S, Liang Marilyn G, Schneider Lynda C
Department of Dermatology, School of Medicine, University of California at Davis, Sacramento, California 95816, USA.
Pediatr Dermatol. 2012 Jan-Feb;29(1):73-8. doi: 10.1111/j.1525-1470.2011.01477.x. Epub 2011 Aug 19.
We sought to describe the clinical outcomes of eight pediatric patients diagnosed with atopic dermatitis (AD) and hypogammaglobulinemia through retrospective review of medical records. All patients presented with severe facial AD. The mean and median ages of diagnosis of hypogammaglobulinemia were 6.2 months and 6.5 months, respectively, with a mean immunoglobulin G (IgG) level of 156 mg/dL. Seven of the eight patients identified in our search demonstrated simultaneous improvement in AD and serum IgG levels within 2 years of initial presentation, suggesting a diagnosis of transient hypogammaglobulinemia. The remaining patient demonstrated normalization by age 6, but no IgG levels had been measured between initial presentation and age 6. The five patients who were tested for specific antibody response to tetanus and Haemophilus influenzae type b vaccination all produced protective responses. All eight patients initially presented with high serum IgE levels. On initial evaluation, three patients had leukocytosis (white blood cell count >18,000 cells/μL), and six had peripheral blood eosinophilia. Three patients outgrew their AD by age 5, and five had clinically good to excellent control of their AD at their last visit, coincident with normalization of IgG levels. Although severe AD and immunoglobulin deficiency may rarely be associated with complex immunodeficiency disorders, our observations suggest that, with careful immunologic monitoring and diligent skin care, most children who present with severe AD and hypogammaglobulinemia exhibit improvement in dermatitis and serum IgG levels within 2 years of onset without major complications.
我们通过回顾病历，试图描述8例诊断为特应性皮炎（AD）和低丙种球蛋白血症的儿科患者的临床结局。所有患者均表现为重度面部AD。低丙种球蛋白血症诊断时的平均年龄和中位年龄分别为6.2个月和6.5个月，平均免疫球蛋白G（IgG）水平为156mg/dL。我们研究中确定的8例患者中有7例在首次就诊后的2年内AD和血清IgG水平同时改善，提示诊断为暂时性低丙种球蛋白血症。其余1例患者在6岁时恢复正常，但在首次就诊至6岁之间未检测IgG水平。对破伤风和b型流感嗜血杆菌疫苗的特异性抗体反应进行检测的5例患者均产生了保护性反应。所有8例患者最初均表现为血清IgE水平升高。初诊时，3例患者有白细胞增多症（白细胞计数>18,000个/μL），6例有外周血嗜酸性粒细胞增多。3例患者在5岁时AD自愈，5例患者在最后一次就诊时AD临床控制良好至极佳，同时IgG水平恢复正常。虽然重度AD和免疫球蛋白缺乏可能很少与复杂的免疫缺陷疾病相关，但我们的观察结果表明，通过仔细的免疫监测和精心的皮肤护理，大多数表现为重度AD和低丙种球蛋白血症的儿童在发病后2年内皮炎和血清IgG水平会有所改善，且无重大并发症。
