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变应性肉芽肿性血管炎的一种局限形式:眼部和皮肤表现。

A limited form of Churg-Strauss syndrome: ocular and cutaneous manifestations.

作者信息

Nissim F, Von der Valde J, Czernobilsky B

出版信息

Arch Pathol Lab Med. 1982 Jun;106(6):305-7.

PMID:6896438
Abstract

A case that supports the concept of a limited form of Churg-Strauss allergic granulomatosis was studied. The patient had a syndrome that was initially confined to the conjunctiva and subsequently displayed cutaneous and subcutaneous lesions. At no time was there any evidence of systemic involvement. Asthma appeared ten years before the onset of the present disease. The characteristic histologic findings consisted of diffuse tissue eosinophilia, and eosinophilic necrotizing and nonnecrotizing granulomatosis. The last, as well as conjunctival involvement, has not been previously described in Churg-Strauss syndrome to our knowledge. Thus, this entity most likely represents a variant of the classic Churg-Strauss syndrome, lacking the multiple organ system involvement that occurs in the latter. The patient responded well to therapy with corticosteroids and as of this writing has been free of disease for about 2 1/2 years.

摘要

对一例支持局限性变应性肉芽肿性血管炎(Churg-Strauss综合征)概念的病例进行了研究。该患者最初表现为仅局限于结膜的综合征,随后出现皮肤和皮下病变。在任何时候均无全身受累的证据。哮喘在当前疾病发作前10年出现。特征性组织学表现包括弥漫性组织嗜酸性粒细胞增多、嗜酸性坏死性和非坏死性肉芽肿形成。据我们所知,后者以及结膜受累在Churg-Strauss综合征中此前未见报道。因此,该病例很可能代表经典Churg-Strauss综合征的一种变异型,缺乏后者所出现的多器官系统受累表现。该患者对皮质类固醇治疗反应良好,截至撰写本文时已无病约2年半。

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