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朗格汉斯细胞组织细胞增生症的多种皮肤表现:一项 10 年回顾性队列研究。

Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study.

机构信息

Dermatology Division, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand.

Clinical Epidemiology Unit, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.

出版信息

Eur J Pediatr. 2019 May;178(5):771-776. doi: 10.1007/s00431-019-03356-1. Epub 2019 Mar 2.

Abstract

Cutaneous manifestation is a common presentation of LCH and is usually a leading clue for the disease diagnosis. Having cutaneous lesions did not show a significantly early age onset at diagnosis compared to those without skin lesions, P value = 0.71. In the present study, cutaneous findings were found as 77.7%. Seborrheic dermatitis-like lesions were the most common cutaneous type (42.8%), followed by papules/nodules/masses (28.5%), petechiae/hemorrhagic lesions (17.8%), and eczematous lesions (10.7%). Time to diagnosis of LCH presented with seborrheic dermatitis-like lesions was significantly longer than other cutaneous presentations, P value = 0.0011.Conclusion: Patients with LCH who had the manifestations of seborrheic dermatitis-like lesions can have diagnosis delayed due to the difficulty in distinguishing these lesions from normal seborrheic dermatitis lesions. Petechiae/hemorrhagic cutaneous signs in addition to the normal seborrheic dermatitis is the clue for early detection of the disease. To improve early detection of LCH, general pediatricians should be alerted to be aware of these skin symptoms, and if they persist, a dermatologist, pediatric if available, should be immediately consulted. What is Known? • Cutaneous manifestation is a common presentation of LCH and is usually a leading clue for the disease diagnosis. What is New? • Patients with LCH who have the manifestations of seborrheic dermatitis-like lesions can have a delayed diagnosis due to the difficulty in distinguishing normal from seborrheic dermatitis lesions. • Petechiae/hemorrhagic cutaneous signs in addition to the normal seborrheic dermatitis are the clue to the early disease detection.

摘要

皮肤表现是 LCH 的常见表现,通常是疾病诊断的主要线索。与无皮肤损伤者相比,有皮肤损伤者在诊断时的年龄无明显提前,P 值=0.71。在本研究中,皮肤表现为 77.7%。脂溢性皮炎样皮损最为常见(42.8%),其次是丘疹/结节/肿块(28.5%)、瘀点/出血性皮损(17.8%)和湿疹样皮损(10.7%)。脂溢性皮炎样皮损患者的 LCH 诊断时间明显长于其他皮肤表现,P 值=0.0011。

结论

患有脂溢性皮炎样皮损的 LCH 患者由于难以将这些皮损与正常脂溢性皮炎皮损区分开来,可能会导致诊断延迟。除了正常的脂溢性皮炎外,瘀点/出血性皮肤表现是早期发现疾病的线索。为了提高 LCH 的早期检出率,普通儿科医生应警惕这些皮肤症状,如果持续存在,应立即咨询皮肤科医生,如有可能,应咨询儿科医生。

已知?

  • 皮肤表现是 LCH 的常见表现,通常是疾病诊断的主要线索。

新发现?

  • 患有脂溢性皮炎样皮损的 LCH 患者由于难以将正常的脂溢性皮炎皮损与脂溢性皮炎样皮损区分开来,可能会导致诊断延迟。

  • 除了正常的脂溢性皮炎外,瘀点/出血性皮肤表现是早期发现疾病的线索。

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