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特发性坏疽性脓皮病与淋巴细胞功能受损:硫唑嘌呤和皮质类固醇治疗失败

Idiopathic pyoderma gangrenosum and impaired lymphocyte function: failure of azathioprine and corticosteroid therapy.

作者信息

Breathnach S M, Wells G C, Valdimarsson H

出版信息

Br J Dermatol. 1981 May;104(5):567-73. doi: 10.1111/j.1365-2133.1981.tb08173.x.

Abstract

A 58-year-old man with chronic "idiopathic' pyoderma gangrenosum, recurrent secondary staphylococcal infection, nail candidiasis, markedly impaired lymphocyte function and a serum blocking factor is described. Despite an initially favourable response to immunosuppressive therapy, the patient later relapsed whilst on high dose systemic corticosteroids and azathioprine. Immune function remains abnormal 3 years cessation of azathioprine. Attempted immunostimulation with Corynebacterium parvum proved unsuccessful. A striking but transient increase in lymphocyte function followed parenteral iron therapy for an unexplained iron-deficiency anaemia. Gradual reduction in prednisone dosage to 5 mg daily has coincided with a period of clinical resolution and modest improvement in lymphocyte reactivity. Prolonged immunosuppressive therapy may be inadvisable in chronic idiopathic pyoderma gangrenosum associated with defective cellular immunity.

摘要

本文描述了一名58岁男性,患有慢性“特发性”坏疽性脓皮病、复发性继发性葡萄球菌感染、指甲念珠菌病、淋巴细胞功能明显受损且存在血清封闭因子。尽管最初对免疫抑制治疗反应良好,但患者在接受高剂量全身性皮质类固醇和硫唑嘌呤治疗时病情复发。停用硫唑嘌呤3年后免疫功能仍异常。用短小棒状杆菌进行免疫刺激未成功。因不明原因的缺铁性贫血接受胃肠外铁剂治疗后,淋巴细胞功能出现显著但短暂的增加。泼尼松剂量逐渐减至每日5毫克,与此同时病情临床缓解,淋巴细胞反应性有适度改善。对于伴有细胞免疫缺陷的慢性特发性坏疽性脓皮病,长期免疫抑制治疗可能不可取。

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