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持久性斑状毛细血管扩张症:不寻常的表现及治疗

Telangiectasia macularis eruptiva perstans: unusual presentation and treatment.

作者信息

Rishpon Ayelet, Matz Hagit, Gat Andrea, Brenner Sarah

机构信息

Department of Dermatology, Tel Aviv-Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

出版信息

Skinmed. 2006 Nov-Dec;5(6):300-2. doi: 10.1111/j.1540-9740.2006.05018.x.

DOI:10.1111/j.1540-9740.2006.05018.x
PMID:17086000
Abstract

A 41-year-old woman presented with a 2-month history of pruritus and a generalized dermatitis that developed initially on the head and spread to the trunk, legs, and buttocks. The pruritus caused extreme discomfort and was not relieved by antihistamines or topical steroid treatment. The patient denied flushing, syncope, and vomiting. Her medical history included asthma treated with salmeterol/fluticasone propionate inhaler, and status post silicone breast augmentation. Physical examination revealed a papular dermatitis on the trunk and extremities composed of lesions up to 0.5 cm in diameter, surrounded by excoriation marks (Figure 1). There was no hepatosplenomegaly or lymphadenopathy. Darier's sign was negative. Results of complete blood count, peripheral blood film examination, and liver function tests were all with normal limits. A biopsy specimen taken from a lesion and stained with hematoxylin-eosin showed telangiectasias, with an increased number of mast cells around blood vessels (Figure 2). Positive Giemsa (Figure 3) and c-kit stain (Figure 4) indicated an increased number of mast cells. Bone marrow aspiration and total body CT performed to rule out systemic involvement showed no pathology. Protein electrophoresis was normal. Serum tryptase and histamine were within normal limits, and 24-hour urine collection for histamine was normal. Narrow-band UV-B treatment was begun 3 times weekly, reduced to twice weekly after 2 months, and then stopped. The first few treatments resulted in significant relief of the pruritus and regression of lesions. After 3 months without treatment, the patient remained free of pruritus and lesions.

摘要

一名41岁女性,有2个月的瘙痒病史及全身性皮疹,最初出现在头部,后蔓延至躯干、腿部和臀部。瘙痒导致极度不适,使用抗组胺药或外用类固醇治疗均未缓解。患者否认有潮红、晕厥和呕吐症状。她的病史包括使用沙美特罗/丙酸氟替卡松吸入器治疗的哮喘,以及硅胶隆胸术后。体格检查发现躯干和四肢有丘疹性皮炎,皮损直径达0.5厘米,周围有抓痕(图1)。无肝脾肿大或淋巴结病。达里埃氏征阴性。全血细胞计数、外周血涂片检查及肝功能检查结果均在正常范围内。取自一处皮损的活检标本经苏木精-伊红染色显示有毛细血管扩张,血管周围肥大细胞数量增加(图2)。吉姆萨染色阳性(图3)和c-kit染色阳性(图4)表明肥大细胞数量增加。为排除全身受累而进行的骨髓穿刺和全身CT检查未发现病变。蛋白电泳正常。血清类胰蛋白酶和组胺在正常范围内,24小时尿组胺收集结果正常。开始每周进行3次窄谱UV-B治疗,2个月后减至每周2次,然后停止治疗。最初几次治疗后瘙痒明显缓解,皮损消退。在未治疗3个月后,患者仍无瘙痒和皮损。

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Telangiectasia macularis eruptiva perstans: more than skin deep.持久性斑疹性毛细血管扩张症:不止于皮肤层面。
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