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一名3个月大婴儿的大疱性肥大细胞增生症。

Bullous mastocytosis in a 3-month-old infant.

作者信息

Asati Dinesh Prasad, Tiwari Anurag

机构信息

Department of Dermatology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India.

Consultant Dermatologist, Centre for Skin Diseases and Laser Treatment, Bhopal, Madhya Pradesh, India.

出版信息

Indian Dermatol Online J. 2014 Oct;5(4):497-500. doi: 10.4103/2229-5178.142520.

DOI:10.4103/2229-5178.142520
PMID:25396140
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4228652/
Abstract

Mastocytosis is a rare myeloid neoplasm characterized by abnormal proliferation and accumulation of mast cells in one or more organ systems including the skin, bone marrow, liver, spleen, lymph nodes and gastrointestinal tract. An infant presenting with bullous lesions is an even rarer clinical presentation of cutaneous mastocytosis. The symptoms and complications are mostly in proportion to the mast cell degranulation in tissues. Management is focused on preventing and treating this event. We report a three-month-old infant with bullous mastocytosis to enhance awareness about this rare diagnosis.

摘要

肥大细胞增多症是一种罕见的髓系肿瘤,其特征是肥大细胞在一个或多个器官系统(包括皮肤、骨髓、肝脏、脾脏、淋巴结和胃肠道)中异常增殖和积聚。一名出现水疱性病变的婴儿是皮肤肥大细胞增多症更为罕见的临床表现。症状和并发症大多与组织中的肥大细胞脱颗粒程度成正比。治疗重点是预防和治疗这一情况。我们报告一名患有水疱性肥大细胞增多症的三个月大婴儿,以提高对这种罕见诊断的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/4d5ca18536ff/IDOJ-5-497-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/9dded9d44a04/IDOJ-5-497-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/2317e59930fc/IDOJ-5-497-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/a38121a868ac/IDOJ-5-497-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/0d249f5ac66a/IDOJ-5-497-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/4d5ca18536ff/IDOJ-5-497-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/9dded9d44a04/IDOJ-5-497-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/2317e59930fc/IDOJ-5-497-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/a38121a868ac/IDOJ-5-497-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/0d249f5ac66a/IDOJ-5-497-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/36a7/4228652/4d5ca18536ff/IDOJ-5-497-g005.jpg

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引用本文的文献

1
Bullous Mastocytosis: A Rare Variant of Diffuse Cutaneous Mastocytosis.大疱性肥大细胞增多症:弥漫性皮肤肥大细胞增多症的一种罕见变体。
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本文引用的文献

1
Diagnosis and treatment of mast cell disorders: practical recommendations.肥大细胞疾病的诊断与治疗:实用建议
Sao Paulo Med J. 2013;131(4):264-74. doi: 10.1590/1516-3180.2013.1314590.
2
Guidelines and diagnostic algorithm for patients with suspected systemic mastocytosis: a proposal of the Austrian competence network (AUCNM).疑似系统性肥大细胞增多症患者的指南和诊断算法:奥地利专业网络(AUCNM)的提议
Am J Blood Res. 2013 May 5;3(2):174-80. Print 2013.
3
Extensive blistering is a predictor for severe complications in children with mastocytosis.
广泛水疱形成是肥大细胞增多症患儿发生严重并发症的一个预测指标。
Allergy. 2012 Oct;67(10):1323-4. doi: 10.1111/all.12013.
4
Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis.血清基础类胰蛋白酶水平升高和广泛皮肤受累是肥大细胞活化症患儿肥大细胞活化症发作严重程度的预测指标。
Allergy. 2012 Jun;67(6):813-21. doi: 10.1111/j.1398-9995.2012.02812.x. Epub 2012 Mar 28.
5
Death from mast cell leukemia: a young patient with longstanding cutaneous mastocytosis evolving into fatal mast cell leukemia.肥大细胞白血病致死:一名长期患有皮肤肥大细胞增多症的年轻患者发展为致命的肥大细胞白血病。
Pediatr Dermatol. 2012 Sep-Oct;29(5):605-9. doi: 10.1111/j.1525-1470.2011.01650.x. Epub 2012 Feb 14.
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Neonatal cutaneous mastocytosis.新生儿皮肤肥大细胞增多症。
J Indian Med Assoc. 2011 May;109(5):354-5.
7
Mast cells as targets of pimecrolimus.肥大细胞作为吡美莫司的作用靶点。
Curr Pharm Des. 2011 Nov;17(34):3823-9. doi: 10.2174/138161211798357827.
8
Diffuse cutaneous mastocytosis with generalized bullae.伴有全身性大疱的弥漫性皮肤肥大细胞增多症。
Ann Dermatol. 2010 Feb;22(1):77-80. doi: 10.5021/ad.2010.22.1.77. Epub 2010 Feb 28.
9
Effects of topical treatment with the raft modulator miltefosine and clobetasol in cutaneous mastocytosis: a randomized, double-blind, placebo-controlled trial.米替福新和氯倍他索乳膏外用治疗皮肤肥大细胞增多症的疗效:一项随机、双盲、安慰剂对照试验。
Br J Dermatol. 2010 Jan;162(1):185-90. doi: 10.1111/j.1365-2133.2009.09434.x. Epub 2009 Aug 3.
10
Pediatric-onset mastocytosis: a long term clinical follow-up and correlation with bone marrow histopathology.儿童期肥大细胞增多症:长期临床随访及与骨髓组织病理学的相关性
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