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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.

DOI:10.1016/s0190-9622(89)70313-3
PMID:2808845
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。

相似文献

1
Idiopathic erythermalgia: a congenital disorder.特发性红斑性肢痛症:一种先天性疾病。
J Am Acad Dermatol. 1989 Nov;21(5 Pt 2):1128-30. doi: 10.1016/s0190-9622(89)70313-3.
2
Cutaneous pathology in primary erythermalgia.原发性红斑性肢痛症的皮肤病理学
Am J Dermatopathol. 1996 Feb;18(1):30-4. doi: 10.1097/00000372-199602000-00005.
3
Secondary erythermalgia in systemic lupus erythematosus; [comment].系统性红斑狼疮中的继发性红斑性肢痛症;[评论]
J Rheumatol. 1993 Jan;20(1):144-6.
4
Erythromelalgia红斑性肢痛症
5
Erythromelalgia and myeloproliferative disorders.红斑性肢痛症与骨髓增殖性疾病。
Arch Intern Med. 1989 Jan;149(1):105-9.
6
[Classification of erythermalgia].[红斑性肢痛症的分类]
J Mal Vasc. 1996;21(2):80-3.
7
Resolution of refractory symptoms of secondary erythermalgia with intermittent epidural bupivacaine.间歇性硬膜外注射布比卡因治疗继发性红斑性肢痛症难治性症状
Reg Anesth Pain Med. 2001 Sep-Oct;26(5):488-90. doi: 10.1053/rapm.2001.25930.
8
[Familial erythermalgia treated with pizotifen].[用苯噻啶治疗家族性红斑性肢痛症]
Ann Dermatol Venereol. 1995;122(11-12):777-9.
9
Secondary erythermalgia associated with an autoimmune disorder of undetermined significance.与意义未明的自身免疫性疾病相关的继发性红斑性肢痛症。
Dermatology. 1995;190(3):232-4. doi: 10.1159/000246692.
10
Thrombocythemia as a cause of erythermalgia.血小板增多症作为红斑性肢痛症的一个病因。
Arch Dermatol. 1977 Apr;113(4):468-71.

引用本文的文献

1
Mutations in sodium-channel gene SCN9A cause a spectrum of human genetic pain disorders.钠通道基因SCN9A的突变会引发一系列人类遗传性疼痛疾病。
J Clin Invest. 2007 Dec;117(12):3603-9. doi: 10.1172/JCI33297.
2
The primary erythermalgia-susceptibility gene is located on chromosome 2q31-32.原发性红斑性肢痛症易感基因位于2号染色体的31-32区。
Am J Hum Genet. 2001 May;68(5):1277-82. doi: 10.1086/320107. Epub 2001 Mar 20.
3
Acute secondary erythermalgia and hypertension in children. Erythermalgia Multidisciplinary Study Group.儿童急性继发性红斑性肢痛症与高血压。红斑性肢痛症多学科研究组
Eur J Pediatr. 1995 Nov;154(11):882-5. doi: 10.1007/BF01957497.
4
Three types of erythromelalgia.三种类型的红斑性肢痛症。
BMJ. 1990 Sep 8;301(6750):454-5. doi: 10.1136/bmj.301.6750.454.