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孤立性黏膜溃疡揭示特发性嗜酸性粒细胞增多综合征

[Isolated mucosal ulcers disclosing idiopathic hypereosinophilic syndrome].

作者信息

Billon C, Gautier C, Villaret E, Ducos M H, Martin J C, Geniaux M

机构信息

Service de Dermatologie, Hôpital Pellegrin, Bordeaux.

出版信息

Ann Dermatol Venereol. 1997;124(3):248-50.

PMID:9686058
Abstract

INTRODUCTION

Idiopathic hypereosinophilic syndrome is an uncommon disease often associated with diverse non-specific skin manifestations. Mucosal ulcerations suggest a myeloproliferative from with poor prognosis due to possible progression to malignant hemopathy or visceral complications.

CASE REPORT

A 28-year-old man presented idiopathic hypereosinophilia with isolated mucosal ulcerations involving the buccal and genital areas. Laboratory results (hematology, CD25) suggested a myeloproliferative form. Treatment with alpha interferon (18 months) led to regression of the mucosal lesions and a decrease in the markers of eosinophil toxicity. There was no visceral involvement.

DISCUSSION

Immunosuppression with/without high-dose alpha interferon is usually used for the treatment of hypereosinophilic syndrome. In our case favorable outcome was obtained with lower doses of alpha interferon than those reported in the literature. There was objective decrease in eosinophil toxicity (regular counts of hypodense eosinophils, CD25 or interleukin 2 soluble receptor) and no progression (malignant hemopathy, mortal visceral involvement).

摘要

引言

特发性嗜酸性粒细胞增多综合征是一种罕见疾病,常伴有多种非特异性皮肤表现。黏膜溃疡提示骨髓增殖性疾病,因其可能进展为恶性血液病或内脏并发症,预后较差。

病例报告

一名28岁男性出现特发性嗜酸性粒细胞增多症,伴有孤立的颊部和生殖器区域黏膜溃疡。实验室检查结果(血液学、CD25)提示为骨髓增殖性疾病。使用α干扰素治疗(18个月)使黏膜病变消退,嗜酸性粒细胞毒性标志物减少。无内脏受累。

讨论

使用或不使用高剂量α干扰素进行免疫抑制通常用于治疗嗜酸性粒细胞增多综合征。在我们的病例中,使用比文献报道剂量更低的α干扰素取得了良好效果。嗜酸性粒细胞毒性客观降低(低密度嗜酸性粒细胞、CD25或白细胞介素2可溶性受体的定期计数),且无进展(恶性血液病、致命性内脏受累)。

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