一例面部难治性肥厚性红斑狼疮,其不可逆的皮肤纤维化在疾病控制下通过局部全厚皮肤移植并联合使用局部和全身免疫抑制剂和羟氯喹进行治疗

IF 1.1 Q4 ALLERGY
Shiori Sekine MD, Shiro Iino MD, Kentaro Nishimura MD, Sayuri Okamura MD, Hiroshi Kasamatsu MD, Noritaka Oyama MD, PhD, Kouichiro Hirai MD, Minoru Hasegawa MD, PhD
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引用次数: 0

摘要

肥厚性红斑狼疮(HLE)是盘状红斑狼疮(DLE)的一种罕见亚型,以疣状和角化过度结节为特征,有影响暴露在阳光下的皮肤的趋势。1它代表了一个波动的临床过程,通常难以保守治疗。2长期病变可能导致美容/功能损伤和过度纤维化,并发展为鳞状细胞癌(SCC),1,3,4,频率未知。目前还没有推荐的治疗方法,但在选定的临床过程中,手术治疗可能会带来一些好处,特别是在皮肤病变发展为不可逆和过度纤维化的情况下。一名60岁的日本男子,其鼻子和嘴唇有30年的反复皮肤侵蚀史,被诊断为DLE(图1A)。皮肤损伤持续存在并逐渐加重。检查显示,鼻背有一个红黄色的不规则肿块,鼻翼和上唇有硬结红斑(图1B、C)。鼻背侧皮肤病理学显示角化过度、毛囊堵塞和基底层局灶性空泡变性,表皮网状嵴明显延长(图1D)。真皮上部有强烈的炎性浸润,主要由淋巴细胞组成。直接免疫荧光在皮-皮交界处对IgG呈阳性(数据未显示)。除此之外,他既没有系统性症状和器官受累,也没有系统性狼疮的证据。根据临床病程和病理,他被诊断为HLE,由先前存在的DLE引起,并给予强效局部皮质类固醇和口服羟氯喹,病情逐渐恶化。同时口服泼尼松(20 mg/天)对皮肤肿块和炎症的进展有效,但未能改善纤维化肿块。由于将SCC的疾病活动和可疑并发症降至最低,我们进行了切除活组织检查,然后使用锁骨上全厚皮片进行重建(图1E)。整个皮肤肿块的组织病理学显示出明显的真皮纤维化,其结果与之前的活检相似,尽管炎症要小得多(图1F)。术后伤口愈合后,局部使用他克莫司治疗残留的DLE病变,成功地将口服泼尼松龙减至2 mg/天,由于对进一步减少剂量有抵抗力,因此继续使用。术后15个月,移植的皮肤逐渐融合周围皮肤的颜色和质地差异(图1G)。肥厚性红斑狼疮仍然是诊断和治疗的挑战。长期病变通常会演变成毁容和筛状疤痕,需要考虑对美容和功能损伤进行替代治疗,5,6就像我们的情况一样。此外,耐药的HLE需要在怀疑时否认恶性肿瘤的可能性。除了组织学证实恶性转化外,手术切除持续存在的HLE皮肤可能有助于去除美容受损、纤维化肿块和潜在的皮肤缺损,以防止复发,特别是面部受累。此外,非暴露皮肤的全厚皮肤移植不仅使受影响的皮肤重新浮出水面,而且在张力和质地上与周围皮肤相匹配。因此,在适当的时间进行手术治疗可能是不可逆性纤维化HLE的可选选择之一,其疾病活动在保守治疗下得到控制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A case of refractory hypertrophic lupus erythematosus on the face whose irreversible skin fibrosis was treated by local full-thickness skin graft under disease control with a combined use of topical and systemic immunosuppressants, and hydroxychloroquine

A case of refractory hypertrophic lupus erythematosus on the face whose irreversible skin fibrosis was treated by local full-thickness skin graft under disease control with a combined use of topical and systemic immunosuppressants, and hydroxychloroquine

Hypertrophic lupus erythematosus (HLE) remains pose a treatment challenge, and often displays disfiguring scar with cosmetic and/or functional impairment. We report a male case of refractory hypertrophic lupus erythematosus on the face, whose disease activity was controlled with a combined use of topical and systemic immunosuppressants, and hydroxychloroquine. The persisted, irreversible fibrotic skin mass was finally treated with surgical approach using full-thickness skin graft, achieving a favorable cosmetic result.

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来源期刊
CiteScore
0.60
自引率
10.00%
发文量
69
审稿时长
12 weeks
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