Kikuchi-Fujimoto disease presenting with papular lesions on the elbows and knees

IF 1.1 Q4 ALLERGY
Izumi Takei MD, Kazuhiro Kawai MD, PhD, Mihoko Yamazaki MD, PhD
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Abstract

Kikuchi-Fujimoto disease is a self-limited febrile lymphadenitis of unknown etiology, possibly associated with cutaneous lesions. We report a case of Kikuchi-Fujimoto disease presenting with papular lesions limited to the elbows and knees. The skin biopsy specimen showed spongiosis and pseudo-Pautrier abscesses in addition to the typical histopathological findings of Kikuchi-Fujimoto disease. Although necrosis was not observed in the lymph node biopsy specimen, histopathological and immunohistochemical findings were consistent with Kikuchi-Fujimoto disease in the proliferating phase.

Abstract Image

菊口-藤本病表现为肘部和膝盖的丘疹性病变
KikuchiFujimoto病(KFD)是一种病因不明的自限性发热性淋巴结炎,主要影响年轻人的颈部淋巴结结节病变表现为皮质旁坏死伴非中性粒细胞核裂,CD8+ T细胞和髓过氧化物酶(MPO)+组织细胞浸润,CD123+浆细胞样树突状细胞簇,尽管根据疾病分期可能没有坏死。据报道,高达40%的病例有皮肤病变皮肤表现多样,但常出现在上半身。我们报告一例KFD与丘疹病变局限于肘部和膝盖。20岁男性,有2周的发热史。体格检查显示双侧后颈、腋窝和腹股沟淋巴结无压痛性淋巴结病变,肘部和膝盖有非瘙痒性红斑丘疹(图1A,B)。实验室检测显示活性蛋白(3.62 mg/dL)和血清乳酸脱氢酶(318 U/L)水平升高;参考范围为124 ~ 222 U/L),白细胞计数降低(1700/μL)。抗核抗体阴性。计算机断层扫描未发现中央淋巴结肿胀。皮肤活检标本显示界面皮炎、乳头状真皮水肿和真皮浸润(图1C)。表皮可见海绵状脓肿和假性脓肿(图1D)。在中性粒细胞缺失的情况下,真皮淋巴组织细胞浸润与核裂有关(图1E)。大多数淋巴细胞CD3阳性,含有CD4+和CD8+细胞,尽管大多数表皮内T细胞是表达TIA1、穿孔素和颗粒酶b的CD8+细胞。真皮浸润含有大量CD68+(克隆KP1和PGM1)组织细胞(图1F),由CD163+、MPO+和CD123+细胞组成。淋巴结活检标本显示在中性粒细胞缺失的情况下,皮质旁淋巴组织细胞浸润伴核裂(图1G)。CD8+ T细胞多于CD4+细胞(图1H,I)。CD68+、CD163+组织细胞丰富,多数表达MPO(图1J、K)。可见CD123+浆细胞样树突状细胞簇(图1L)。虽然在淋巴结活检标本中未观察到坏死,但根据组织病理学和免疫组织化学结果诊断为增殖期KFD。1,2,4 - 6仅给予口服非甾体抗炎药和外用皮质类固醇的支持治疗。发热、淋巴结病变和皮肤病变在发病后5周内自行消退。KFD的皮肤病变表现为真皮浅层和深层淋巴组织细胞浸润、非中性粒细胞核裂、界面皮炎和乳头状真皮水肿。虽然在一些病例中观察到海绵状病变,但假性肛周脓肿尚未见报道。本病例中皮肤病变局限于肘部和膝关节的原因尚不清楚,但其定位和海绵状表皮变化提示外源性刺激的参与。KFD的鉴别诊断主要包括SLE和淋巴瘤在我们的病例中,血清抗核抗体为阴性,除了发烧和淋巴结病外,没有提示SLE的临床体征。组织病理学和免疫组织化学
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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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