Linear scleroderma en coup de sabre - a different clinical presentation.

IF 1 4区 医学 Q4 RHEUMATOLOGY
Acta reumatologica portuguesa Pub Date : 2021-01-01
Miguel Vieira Martins, Isabel Azevedo, Carlos Rodrigues, Margarida Oliveira
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引用次数: 0

Abstract

Localized scleroderma (LoS) is a rare condition featuring skin and underlying tissue sclerosis not usually compromising other systems. A subtype of LoS including lesions in the head is further classified as linear scleroderma en coup de sabre (LSeCS). Neurological involvement in LSeCS can reach up to 4% and may include seizures. Cutaneous lesions usually emerge before neurologic symptoms and these oftentimes manifest with intracranial abnormalities. We describe a case of an 11-year-old boy with an onset of self-limited unexplained seizures at 20-months of life. During the first year of follow-up, a midline frontoparietal lesion with alopecia and hypopigmentation was noted and a referral to dermatology and pediatric rheumatology consultation was made. A diagnosis of LEsCS was made. A 10-year follow-up of this patient is presented with favorable outcome. LSeCS is a rare form of LoS most frequently diagnosed in children and adolescents. A meticulous examination of these patients should be performed with particular attention to the face and scalp. The mainstay therapeutical approach is based on methotrexate and corticosteroids. Neurologic abnormalities associated with skin lesions on the head should should raise clinical suspicion of LSeCS.

线性硬皮病偶发-不同的临床表现。
局部硬皮病(LoS)是一种罕见的疾病,其特征是皮肤和底层组织硬化,通常不会损害其他系统。包括头部病变的一种LoS亚型被进一步归类为线状刀发硬皮病(LSeCS)。LSeCS的神经系统受累率高达4%,可能包括癫痫发作。皮肤病变通常出现在神经症状之前,这些症状通常表现为颅内异常。我们描述了一个11岁的男孩在20个月大时出现自限性不明原因癫痫发作的病例。在随访的第一年,注意到前额顶叶中线病变伴有脱发和色素沉着减退,并转介到皮肤科和儿科风湿病学会诊。诊断为LEsCS。对该患者进行了10年的随访,结果良好。LSeCS是一种罕见的LoS形式,最常见于儿童和青少年。对这些患者应进行细致的检查,特别注意面部和头皮。主要的治疗方法是基于甲氨蝶呤和皮质类固醇。与头部皮肤病变相关的神经系统异常应引起临床对LSeCS的怀疑。
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来源期刊
Acta reumatologica portuguesa
Acta reumatologica portuguesa 医学-风湿病学
自引率
0.00%
发文量
0
审稿时长
>12 weeks
期刊介绍: Acta Reumatólogica Portuguesa is a scientific peer reviewed journal covering all aspects of rheumatic diseases and related to Rheumatology. The journal publishes original articles, reviews, clinical cases, images in rheumatology, letters to the editor and clinical teaching (e.g. guidelines and clinical protocols). Published since 1973, Acta Reumatológica Portuguesa is the official scientific publication of the Portuguese Society of Rheumatology, a non-profit organization that promotes the knowledge and investigation of rheumatic diseases and the development of Rheumatology.
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