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[皮肤血管炎、骨髓增生异常综合征和复发性多软骨炎]

[Cutaneous vasculitis, myelodysplasia and relapsing polychondritis].

作者信息

Németh-Normand F, Machet L, Vaillant L, Fontes V, Lefrancq T, Lorette G

机构信息

Service de Dermatologie, CHU Trousseau, route de Loches, 37044 Tours Cedex, France.

出版信息

Ann Dermatol Venereol. 2002 Nov;129(11):1299-302.

Abstract

BACKGROUND

Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved.

CASE REPORT

We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia.

DISCUSSION

Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.

摘要

背景

复发性多软骨炎是一种罕见的自身免疫性疾病,其特征为软骨组织反复发生炎症。在某些情况下,许多其他组织也可能受累。

病例报告

我们描述了一名64岁复发性多软骨炎男性患者的病例,其最初症状为丘疹结节性和黏膜阿弗他样病变。皮肤活检显示为无白细胞破碎性特征的血管炎。仅在两年后,由于反复出现耳软骨炎,才确诊为复发性多软骨炎。自疾病开始以来,就伴有骨髓发育异常。

讨论

在复发性多软骨炎的病程中,不到50%的病例会出现皮肤表现。已有报告称其与骨髓发育异常有关,这可能是复发性多软骨炎的一种特殊形式。骨髓发育异常综合征与复发性多软骨炎的关联比与其他血管炎更为常见。特别是当存在皮肤和黏膜表现时,必须进行排查。

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