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自愈性青少年皮肤粘蛋白病:中东地区一例报告

Self-Healing Juvenile Cutaneous Mucinosis: A Case Report in the Middle East.

作者信息

Geagea Caroline, Youssef Nour, Wakim Gerard

机构信息

Department of Pediatrics and Adolescent Medicine, Lebanese American University Medical Center, Beirut, Lebanon.

出版信息

Am J Case Rep. 2019 Jan 16;20:65-69. doi: 10.12659/AJCR.910047.

DOI:10.12659/AJCR.910047
PMID:30647403
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6345112/
Abstract

BACKGROUND Self-healing juvenile cutaneous mucinosis (SHJCM) is a rarely diagnosed disease worldwide, with less than 20 reported cases in the literature. It is characterized by a rather benign course in juvenile patients with nodular and mucinous skin eruption and edema. CASE REPORT A 12-year-old male patient previously healthy presented to the pediatrics clinic with a 1-week history of bilateral palmer pruritus and plantar tenderness upon walking, preceded by eruption of erythematous patch on his neck. The disease course evolved to include facial edema, erythema, nodular skin eruptions with a completely negative initial workup. The patient was labelled as a juvenile idiopathic arthritis patient and doomed to be a candidate for corticosteroid therapy. Upon further workup, a skin biopsy was taken and SHJCM was diagnosed. Complete resolution of symptoms was witnessed on symptomatic treatment after 5 months of diagnosis. CONCLUSIONS To our knowledge, this is the second case of SHJCM reported in the Middle East and the first to be reported in Lebanon. It is also the first case reported to have the longest follow-up period; 10 years of follow-up with no new findings or relapse. SHJCM is a rare disease whereby awareness of its features and presentation may help in diagnosing it and preventing unnecessary testing and aggressive treatment for a rather benign disease.

摘要

背景

自愈性青少年皮肤黏蛋白病(SHJCM)在全球范围内是一种罕见的诊断疾病,文献报道的病例少于20例。其特征是在青少年患者中病程较为良性,伴有结节性和黏液性皮肤疹及水肿。

病例报告

一名12岁既往健康的男性患者到儿科门诊就诊,有双侧手掌瘙痒1周病史,行走时足底压痛,之前颈部出现过红斑。疾病进程发展为包括面部水肿、红斑、结节性皮肤疹,初始检查结果完全为阴性。该患者被诊断为青少年特发性关节炎患者,注定要成为皮质类固醇治疗的候选者。经过进一步检查,进行了皮肤活检,诊断为SHJCM。诊断后5个月对症治疗后症状完全缓解。

结论

据我们所知,这是中东地区报道的第二例SHJCM病例,也是黎巴嫩报道的首例。这也是报道的随访期最长的病例;随访10年无新发现或复发。SHJCM是一种罕见疾病,了解其特征和表现可能有助于诊断该病,并避免对一种相当良性的疾病进行不必要的检查和积极治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/db8f7d38dfd1/amjcaserep-20-65-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/bf701e25eaa5/amjcaserep-20-65-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/d3194e7481c3/amjcaserep-20-65-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/819d5b3348ab/amjcaserep-20-65-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/5a3e5c7197d8/amjcaserep-20-65-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/db8f7d38dfd1/amjcaserep-20-65-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/bf701e25eaa5/amjcaserep-20-65-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/d3194e7481c3/amjcaserep-20-65-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/819d5b3348ab/amjcaserep-20-65-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/5a3e5c7197d8/amjcaserep-20-65-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ae86/6345112/db8f7d38dfd1/amjcaserep-20-65-g005.jpg

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Self-healing juvenile cutaneous mucinosis: challenging diagnosis and management.自愈性青少年皮肤粘蛋白病:具有挑战性的诊断与管理
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