成人起病的朗格汉斯细胞组织细胞增多症:一例临床病例

O. Chernysheva, O. Dorokhina, A. Khlebnikova, E. Selezneva
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引用次数: 1

摘要

朗格汉斯细胞组织细胞增多症主要诊断于儿童,其在成年期的表现相当罕见。皮疹可能是非特异性的,类似于许多皮肤病。因此,临床诊断是具有挑战性的,作为一个规则,这样的病人被误解,并管理为其他疾病由皮肤科医生数年。我们报告一个35岁女性患者的朗格汉斯细胞组织细胞增多症伴皮肤累及的临床病例,她曾因脓皮病、脂溢性皮炎和皮肤褶念珠菌病接受皮肤科医生治疗2年。考虑到临床体征和症状以及表现的年龄,我们最初怀疑家族性良性天疱疮(Hailey-Hailey病)或滤泡性角化不良(Darier病)。然而,组织学评估显示朗格汉斯细胞组织细胞增生,免疫组织化学证实抗朗格汉斯素,抗cd1a和抗蛋白S-100抗体。患者被转诊到血液科接受进一步的检查和特异性治疗。在任何治疗难治性疾病的情况下,对长期常规治疗没有反应,有必要通过组织学检查重新考虑诊断。它将允许识别疾病,这是罕见的皮肤科实践。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Adult-onset of Langerhans cell histiocytosis: a clinical case
Langerhans cell histiocytosis is mainly diagnosed in children, and its manifestation in adult age is quite uncommon. Skin rashes may be non-specific and mimic a number of dermatoses. Therefore, the clinical diagnosis is challenging and as a rule, such patients are misinterpreted and managed for other disorders by a dermatologist for some years. We present a clinical case of Langerhans cell histiocytosis with skin involvement in a 35-year female patient, who had been treated by a dermatologist for 2 years for pyoderma, seborrheic dermatitis, and skin fold candidiasis. Taking into account the clinical signs and symptoms and age of manifestation, we initially suspected familial benign pemphigus (Hailey-Hailey disease) or follicular dyskeratosis (Darier's disease). However, the histological assessment showed Langerhans cell histiocytosis confirmed by immunohistochemistry with anti-langerin, anti-CD1a, and anti-protein S-100 antibodies. The patient was referred to a hematologist for further work-upand specific treatment. In cases of any treatment resistant disorders, which do not respond to long-term conventional treatment, it is necessary to reconsider the diagnosis by means of histological investigation. It would allow for identification of a disease, which is uncommon in dermatology practice.
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