免疫血清球蛋白引發全身過敏性血管炎

Systemic Hypersensitivity Vasculitis after Receiving Immune Serum Globulin-- A Case Report

全身過敏性血管炎臨床上表現於皮膚症狀，不同程度的器官系統牽連較少見。導致過敏性血管炎的抗原可能是動物血清，藥物，化合物，微生物甚至源於人類的蛋白質。一位十五歲的女孩接受免疫血清球蛋白注射後，引起全身過敏性血管炎。經過臺大醫院五個月的類固醇治療，最後死於多重器官衰竭。病理解剖時全身組織有小靜脈及微血管壁的淋巴球浸潤，皮膚、腎臟、肝臟、脾臟、胰臟、腸管、肺臟、甲狀腺及腦膜均可發現。皮膚的免疫螢光研究發現IgG、Clg及C4的顆粒狀沉積於血管壁上。

Although various degrees of systemic involvement occur in hypersensitivity vasculitis, usually the cutaneous involvement is the main manifestation. An autopsy-proved case of systemic hypersensitivity vasculitis with multiple organ involvement is reported herein. A 15-year-old girl received immune serum globulin injection under impression of German measles. A week later, skin lesions became worse and high fever, generalized arthralgia, bloody diarrhea and hematuria were noted. Hypocomplementemia was detected; other immunological tests including ANA, LE cell, RF, anti-ds DNA and anti- ENA were all negative. After corticosteroid treatment, skin rash disappeared and fever subsided. Owing to subsequent lung edema, pulse therapy was tried. However, systemic fungal infection developed and Aspergillus fumigatus was isolated. Multiple organ failure took an irreversible course, and she expired after five- months' admission. At autopsy, lymphocyte infiltration was found predominantlf along venous channels and capillaries in multiple organs including skin, kidney, liver, spleen, pancrease, intestine, lungs, thyroid and leptomeninges. Immunoflaorescence study of skin revealed granular deposits of IgG, Clq and C4 along the vessel walls.