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自愈性幼年皮肤黏液瘤病,一种类似幼年皮肌炎的硬皮病样紊乱:基于病例的综述。

Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review.

机构信息

Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.

Department of Pediatric Clinical Immunology and Rheumatology, Allergy Immunology Unit, Postgraduate Institute of Medical Education and Research, Advanced Pediatric Center, Chandigarh, 160012, India.

出版信息

Rheumatol Int. 2020 Nov;40(11):1911-1920. doi: 10.1007/s00296-020-04578-5. Epub 2020 Apr 22.

Abstract

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare childhood disease with characteristic cutaneous and rheumatic manifestations. Cutaneous manifestations include a combination of nodules affecting peri-articular (especially interphalangeal joints) and head and neck areas; and linearly arranged ivory white papules over an erythematous indurated skin. Despite a benign course, an abrupt onset of symptoms with extensive cutaneous involvement often leads to parental anxiety, overenthusiastic evaluation and sometimes aggressive treatment. A peculiar cutaneous distribution in SHJCM including nodular lesions and periorbital edema, arthritis and arthralgia in a few cases, may simulate juvenile dermatomyositis. It is, therefore, important for dermatologists and pediatricians to be aware of this entity. In this report, we describe two cases of SHJCM and briefly review similarly reported cases in children.

摘要

自限性幼年皮肤黏液瘤病(SHJCM)是一种罕见的儿童疾病,具有特征性的皮肤和风湿表现。皮肤表现包括影响关节周围(特别是指间关节)和头颈部的结节的组合;以及在红斑性硬结皮肤上呈线性排列的象牙白色丘疹。尽管病程良性,但广泛的皮肤受累突然发作往往会导致父母焦虑、过度评估,有时甚至是激进治疗。SHJCM 的一种特殊皮肤分布包括结节性病变和眶周水肿、少数情况下的关节炎和关节痛,可能类似于幼年皮肌炎。因此,皮肤科医生和儿科医生了解这一实体非常重要。在本报告中,我们描述了两例 SHJCM 病例,并简要回顾了儿童中类似报道的病例。

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