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[伴有大量水疱的弥漫性皮肤肥大细胞增多症的良性病程]

[Benign course of diffuse cutaneous mastocytosis with massive blisters].

作者信息

Zink A, Grosber M, Schuch A, Biedermann T, Brockow K

机构信息

Klinik und Poliklinik für Dermatologie und Allergologie am Biederstein, Klinikum rechts der Isar, Technische Universität München, Biedersteiner Str. 29, 80802, München, Deutschland.

出版信息

Hautarzt. 2015 Sep;66(9):691-3. doi: 10.1007/s00105-015-3612-2.

Abstract

BACKGROUND

Diffuse cutaneous mastocytosis is a rare disease with increased numbers of mast cells and development of blisters, which can be easily overlooked.

CASE REPORT

A 6-month-old girl was presented by her parents with acute onset of numerous, disseminated bullae on her body. Histology revealed numerous mast cells in a skin sample and highly elevated serum tryptase levels were detected. The diagnosis of diffuse cutaneous mastocytosis was made. The patient was medically treated with glucocorticoids and antibiotics. Within a few years of time a complete remission of developing bullae, a major clinical improvement as well as a continuous decrease of basal tryptase was seen. Today, the girl is 14 years old and without any apparent limitation due to the disease and in fact she is very successful in competitive sports.

CONCLUSION

Despite often severe symptoms at first manifestation, this clinical development showing a benign course is typical in children.

摘要

背景

弥漫性皮肤肥大细胞增多症是一种罕见疾病,其特征为肥大细胞数量增加并出现水疱,很容易被忽视。

病例报告

一名6个月大的女童被父母带到医院,其身上急性出现大量散发的水疱。组织学检查显示皮肤样本中有大量肥大细胞,并且检测到血清类胰蛋白酶水平显著升高。由此诊断为弥漫性皮肤肥大细胞增多症。该患者接受了糖皮质激素和抗生素治疗。几年内,水疱发展完全缓解,临床症状显著改善,基础类胰蛋白酶水平持续下降。如今,这个女孩14岁,并未因该疾病受到任何明显限制,事实上她在竞技体育方面非常成功。

结论

尽管最初表现往往症状严重,但这种呈现良性病程的临床发展在儿童中很典型。

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