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特发性红斑性肢痛症:一种先天性疾病。

Idiopathic erythermalgia: a congenital disorder.

作者信息

Michiels J J, van Joost T, Vuzevski V D

机构信息

Department of Hematology, University Hospital Dijkzigt, Erasmus University, Rotterdam, The Netherlands.

出版信息

J Am Acad Dermatol. 1989 Nov;21(5 Pt 2):1128-30. doi: 10.1016/s0190-9622(89)70313-3.

Abstract

Idiopathic erythermalgia during early childhood and adolescence is characterized by red, congested, burning pain of the lower extremities provoked by exercise or exposure to warmth. The clinical symptoms of idiopathic erythermalgia in a young woman and her mother are described. Histopathologic and immunofluorescence findings in biopsy specimens of affected areas of skin were consistent with a nonspecific inflammatory process. The condition was completely refractory to any treatment. Even the long-lasting relief of pain with one low dose of aspirin, which is a prerequisite for the diagnosis of thrombocytemic erythromelalgia, was lacking. Idiopathic erythermalgia appears to be a separate clinical entity and congenital disorder.

摘要

儿童期和青春期特发性红斑性肢痛症的特征是运动或暴露于温暖环境时下肢出现发红、充血、灼痛。本文描述了一名年轻女性及其母亲特发性红斑性肢痛症的临床症状。皮肤受累区域活检标本的组织病理学和免疫荧光结果与非特异性炎症过程一致。该病症对任何治疗均完全无效。甚至连小剂量阿司匹林能带来持久疼痛缓解这一血小板减少性红斑性肢痛症诊断的先决条件也不具备。特发性红斑性肢痛症似乎是一种独立的临床实体和先天性疾病。

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