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环状毛细血管扩张:持久性斑状毛细血管扩张症的一种不寻常表现。

Ringed telangiectasias: an unusual presentation of telangiectasia macularis eruptiva perstans.

作者信息

Siddique Laila, Ahrns Hadjh, Seiverling Elizabeth

机构信息

Penn State College of Medicine, Penn State Hershey Medical Center, Hershey, Pennsylvania, USA.

Department of Family and Community Medicine, Penn State Hershey Medical Center, Hershey, Pennsylvania, USA.

出版信息

BMJ Case Rep. 2017 Nov 30;2017:bcr-2017-221784. doi: 10.1136/bcr-2017-221784.

Abstract

Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis. While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. TMEP is typically characterised by scattered red-brown macules on the trunk and extremities, but must be differentiated from other telangiectatic conditions such as scleroderma, hereditary haemorrhagic telangiectasia and telangiectasias secondary to cirrhosis. Practitioners must be aware that variants to the classic presentation of TMEP exist, such as the ringed telangiectasias we describe. Diagnostic workup including tissue biopsy must be considered in such patients after a thorough history and physical have been performed and other telangiectatic processes have been ruled out. The treatment of cutaneous mastocytosis aims at controlling symptoms and preventing mast cell degranulation. Cosmetic treatment includes the use ofPsoralen and ultraviolet A (PUVA) therapy, total skin electron beam radiation and flashlamp pulsed-dye laser treatment.

摘要

持久性发疹性斑状毛细血管扩张症(TMEP)是一种罕见的皮肤肥大细胞增多症。大多数皮肤肥大细胞增多症发生于儿童且无症状,而TMEP主要发生于成人,并伴有全身表现,需要药物治疗。TMEP的典型特征是躯干和四肢出现散在的红棕色斑疹,但必须与其他毛细血管扩张性疾病相鉴别,如硬皮病、遗传性出血性毛细血管扩张症以及肝硬化继发的毛细血管扩张症。从业者必须意识到TMEP存在经典表现的变体,如我们所描述的环状毛细血管扩张症。在对这类患者进行全面的病史询问和体格检查并排除其他毛细血管扩张性疾病后,必须考虑进行包括组织活检在内的诊断性检查。皮肤肥大细胞增多症的治疗旨在控制症状并防止肥大细胞脱颗粒。美容治疗包括使用补骨脂素和紫外线A(PUVA)疗法、全身皮肤电子束放射治疗以及闪光灯脉冲染料激光治疗。

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Cutaneous mastocytosis: a review focusing on the pediatric population.皮肤肥大细胞增多症:聚焦儿科人群的综述
Clin Pediatr (Phila). 2008 Oct;47(8):757-61. doi: 10.1177/0009922808318344. Epub 2008 May 23.
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Systemic mastocytosis.系统性肥大细胞增多症
Annu Rev Med. 2004;55:419-32. doi: 10.1146/annurev.med.55.091902.103822.
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Telangiectasia macularis eruptiva perstans.持久性斑状毛细血管扩张症
Pediatr Dermatol. 2000 May-Jun;17(3):194-7. doi: 10.1046/j.1525-1470.2000.01750.x.

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