一名儿科女病人的嗜酸性细胞性蜂窝织炎(韦尔斯综合征)。

Pub Date : 2024-08-01 Epub Date: 2024-03-21 DOI:10.5546/aap.2023-10187.eng
María J Aráoz, María M Buján, María Del V Centeno, Andrea B Cervini
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引用次数: 0

摘要

韦尔斯综合征或嗜酸细胞性蜂窝织炎是一种原因不明的炎症性疾病,在儿童中并不常见。临床上通常表现为红斑、水肿性斑块、结节、丘疹、水疱等症状。在此,我们描述了一例女性儿童患者的病例,她患有全身性、无症状的皮下结节,并伴有严重的嗜酸性粒细胞增多。病变的组织病理学检查与威尔斯综合征相符。为了确定病因并寻找相关的嗜酸性粒细胞疾病,对患者进行了跨学科评估;评估结果为阴性。患者接受了全身皮质类固醇激素治疗,反应良好;然而,鉴于停药后皮损复发,患者接受了地塞米松作为二线治疗,随后皮损和嗜酸性粒细胞增多情况有所改善。本报告旨在描述一名女性威尔斯综合征患者的非典型表现以及由此带来的治疗挑战。
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Eosinophilic cellulitis (Wells' syndrome) in a female pediatric patient.

Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.

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