Kufner A, Fritsch P
Hautarzt. 1985 May;36(5):274-80.
Allergic granulomatosis is a rare life-threatening systemic disorder of unknown origin, which represents a variant of systemic necrotizing vasculitis affecting medium-sized arteries and venules. Histologically, allergic granulomatosis is characterized by vascular and extravascular necrotizing palisading granulomas with prominent eosinophilia (Churg-Strauss granulomas). The clinical criteria include atopy with severe allergic asthma, pronounced peripheral eosinophilia, and nodular infiltrates of the skin and internal organs (Churg-Strauss granulomas). The internal organs most commonly involved are the lungs, gastrointestinal tract and, less often, the peripheral nerves, heart, and kidneys. Associated symptoms include fever, arthralgias and skin rashes, such as erythema multiforme, necrotizing venolitis, fixed drug eruption, and urticaria. Allergic granulomatosis shares common features with Wegener's granulomatosis and polyarteritis nodosa and may be related to the latter condition; overlap syndromes are a well-known occurrence. Similar to the other manifestations of systemic necrotizing vasculitis, immune complexes have been detected in fresh lesions and are suspected of being the basic pathogenetic findings. The causative antigens are likely to be respiratory antigens. The prognosis of untreated allergic granulomatosis is poor (mortality of approximately 50% within the first year). Systemic corticosteroids and cyclophosphamide are effective; complete remissions following cyclophosphamide treatment have been reported.
变应性肉芽肿病是一种罕见的、危及生命的、病因不明的全身性疾病,是系统性坏死性血管炎的一种变体,累及中动脉和小静脉。组织学上,变应性肉芽肿病的特征是血管性和血管外坏死性栅栏状肉芽肿,伴有显著的嗜酸性粒细胞浸润(Churg-Strauss肉芽肿)。临床标准包括伴有严重过敏性哮喘的特应性、明显的外周嗜酸性粒细胞增多以及皮肤和内脏的结节性浸润(Churg-Strauss肉芽肿)。最常受累的内脏器官是肺、胃肠道,较少累及外周神经、心脏和肾脏。相关症状包括发热、关节痛和皮疹,如多形红斑、坏死性静脉炎、固定性药疹和荨麻疹。变应性肉芽肿病与韦格纳肉芽肿病和结节性多动脉炎有共同特征,可能与后者有关;重叠综合征很常见。与系统性坏死性血管炎的其他表现相似,在新鲜病变中已检测到免疫复合物,怀疑其为基本的发病机制。致病抗原可能是呼吸道抗原。未经治疗的变应性肉芽肿病预后较差(第一年死亡率约为50%)。全身用皮质类固醇和环磷酰胺有效;有报道称环磷酰胺治疗后可完全缓解。