Bronchial Asthma and Septic Lung in a Patient with Hyperimmunoglobulin E Syndrome

高免疫球蛋白E症候群併發氣喘與敗血性肺炎：病例報告

歐軒甫(Hsuan-Fu Ou) ； 謝俊民(Jiunn-Min Shieh) ； 柯獻欽(Shian-Chin Ko)

《胸腔醫學》 29卷1期 (2014/02) Pp. 39-45

摘要

高免疫球蛋白E症候群是一種先天性、罕見的免疫功能缺陷疾病、可以體染色體顯性或隱性方式遺傳，二者在臨床上表現不同。高免疫球蛋白E症候群最常見的表現為復發性的皮膚感染、復發性肺炎伴隨肺囊腫的形成、與血清免疫球蛋白E濃度增高，顯性遺傳患者常合併臉部、牙齒及骨骼特徵，如：臉型粗獷、皮膚粗糙、額頭明顯、下巴前凸、鼻翼距離增加、乳牙不易脫落等。皮膚特徵包括：異位性皮膚炎與間歇性葡萄球菌膿瘍。復發性感染為此病的主要特色，特別是金黃色葡萄球菌與黴菌感染。處置上首重感染的預防與治療，減少合併症的產生，藥物包括：抗生素、抗黴菌劑、與抗病毒劑。本文報告一例高免疫球蛋白E症候群併發氣喘、反覆性菌血症、細菌性骨髓炎、與敗血性肺炎，長期抗生素治療合併脊椎手術，終使病人順利出院。高免疫球蛋白E症候群影響多個器官系統，故需各個相關學科協調處置，方可使病人得到最好的照顧。

關鍵字

高免疫球蛋白E症候群 ； 敗血性肺炎 ； 葡萄球菌感染

並列摘要

Hyperimmunoglobulin E syndrome (HIES), or Job's syndrome, is a rare and complex primary immunodeficiency disorder characterized by a spectrum of abnormalities related to the immune system, connective tissue, and bones. Despite more than 40 years of research, the etiology of HIES is still unclear. Most cases are sporadic, but autosomal dominant (AD) and autosomal recessive (AR) inheritances have been reported. HIES is characterized by a particular susceptibility to staphylococcal and mycotic infections. Therapy for HIES is directed at prevention and management of infections through the use of sustained systemic antibiotics and antifungals along with topical therapy for eczema and drainage of abscesses. Anti-staphylococcal antibiotic prophylaxis is useful. We present the case of a patient with HIES syndrome who had a long history of bronchial asthma. She was admitted due to recurrent oxacillin-resistant Staphylococcus aureus (ORSA) bacteremia, infectious spondylodiscitis (L2-3) and septic lungs.

並列關鍵字

hyperimmunoglobulin E syndrome (HIES) ； bronchial asthma ； staphylococcus aureus infection