IgG4相关皮肤病伴小腿斑块1例

IF 1.1 Q4 ALLERGY

Journal of Cutaneous Immunology and Allergy Pub Date : 2023-06-04 DOI:10.1002/cia2.12309

Natsumi Fushida MD, Yoshinobu Okamoto MD, PhD, Yasuhito Hamaguchi MD, PhD, Takashi Matsushita MD, PhD

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引用次数: 0

摘要

A.69-年-老的成年男子是参考到我们的医院具有一10-年历史属于非乳白色的棕色的斑块在…上这个降低腿。皮检查揭示一软的棕色的匾牌（30毫米在里面直径）在…上这个正确的降低腿和棕色-有色的硬化的匾牌测量100毫米在…上这个专业轴属于这个左边一（图1A、B）。不理智的分析揭示稠密的渗透淋巴细胞和血浆单元格围绕血船只和脂肪隔膜和标记纤维化延伸从…起这个粗略的真皮到皮下的富含脂肪的组织（图1D、E）。更多比 70% 属于免疫球蛋白 G （IgG）+血浆单元格是建立到是IgG4-生产（图1F，G）。此外抹除的静脉炎是观察到的在里面这个真皮（图1H，I）。不淋巴毛囊组成或嗜酸性粒细胞增多是观察到。实验室测验后果显示高贵的级别属于IgG4（186毫升/分升；典型的4.5–117mg/dL）。已计算层析成像（CT）揭示低-密集区域周围的这个胸部脊柱和腹部的主动脉（图1J，K），没有头和脖子病变。这个病人是已诊断具有IgG4-相关的病（IgG4-RD）相符合的到这个综合的诊断标准提议的通过Umeharaet第1页口头的管理属于皮质类固醇（30毫克/天）是已启动和 IgG4 数量是归一化的 1个月之后治疗（86.5 mg/dL）。沿着具有递减 IgG4 级别，这个斑块逐步地褪色的和压扁的（图1C），和这个低- 密集区域周围的这个胸部脊柱和腹部的主动脉逐步地减少。泼尼松龙是锥形的和维护在一剂量属于9mg/天，没有复发。IgG4-RD是一系统的煽动性的病具有特征的通过高贵的级别属于血清IgG4和渗透属于IgG4-积极乐观的血浆单元格和组织纤维化。2一些病人具有 IgG4-RD展览皮病变，哪一个登仓 et 等。定义像 IgG4-相关的皮病（IgG4-RSD）.3 它通常地清单像瘙痒的结节，丘疹，脓疱，和斑块，哪一个是主要地定位在…上这个头和颈部。4在里面病人具有IgG4-RSD，大的斑块在…上这个四肢有不曾经据报道。在此我们描述一稀有的案例属于 IgG4-RSD具有一大的匾牌属于100毫米在…上这个专业轴属于这个降低腿自从有关时事的类固醇有较少的效应在…上IgG4-RSD，口头的管理属于皮质类固醇是这个现在的第一-线治疗对于另外器官像好这个组织学的功能属于IgG4-RSD相异轻微地从…起那些属于IgG4-RD。关于IgG4-RD，这个数字属于IgG4+血浆单元格每高-权力领域（HPF）是经常≥50然而在里面病人具有 IgG4-RSD，这些血浆单元格很少地超过 50 纤维化是经常较温和的在里面病人具有 IgG4-RSD 比在里面那些具有 IgG4-RD。此外，抹除的静脉炎有很少地曾经已报告在里面病人具有 IgG4-RSD。在里面我们的案例我们建立更多比60 IgG4+血浆单元格每HPF和观察到的标记纤维化和抹除的静脉炎，哪一个是一稀有的案例具有非典型的功能。虽然这个高的数量属于纤维化在里面这病人也许有曾经引起的通过慢性的发炎对于10年，这个准确的机制保持不清楚。我们计划到小心班长这病人对于复发虽然锥形泼尼松。

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A case of IgG4-related skin disease with plaques on the lower legs

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A case of IgG4-related skin disease with plaques on the lower legs

IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by elevated levels of serum IgG4 and infiltration of IgG4-positive plasma cells and tissue fibrosis. Some patients with IgG4-RD exhibit skin lesions, and this was defined as IgG4-related skin disease (IgG4-RSD). Here, we report a case of IgG4-RSD with plaques on the lower legs.

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来源期刊

Journal of Cutaneous Immunology and Allergy Medicine-Dermatology

CiteScore

0.60

自引率

10.00%

发文量

审稿时长

12 weeks