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173例儿童皮肤肥大细胞增多症的临床表现

Presentation of cutaneous mastocytosis in 173 children.

作者信息

Hannaford R, Rogers M

机构信息

New Children's Hospital, Sydney, New South Wales, Australia.

出版信息

Australas J Dermatol. 2001 Feb;42(1):15-21. doi: 10.1046/j.1440-0960.2001.00466.x.

Abstract

This is a retrospective review of the case files and clinical photographs of 173 children diagnosed with cutaneous mastocytosis by a dermatologist in an exclusively paediatric practice. Of the 98 males and 75 females, 51% had mastocytomas, 47% had urticaria pigmentosa and three patients had diffuse cutaneous mastocytosis. Of these cases, 87% first appeared prior to or at 6 months of age. Flushing occurred in 26% of urticaria pigmentosa cases, 29% of mastocytomas and 100% of diffuse cutaneous mastocytosis. Blistering was noted in 23% of urticaria pigmentosa, 31% of mastocytomas and 100% of diffuse cases. Neither symptom was noted in 59% of urticaria pigmentosa and 49% of mastocytomas. There were three cases with a positive family history. The finding of a palmar mastocytoma has only once been previously reported. Illustrated descriptions of our cases are provided.

摘要

这是一项对病例档案和临床照片的回顾性研究,研究对象为一位儿科专科医生诊断为皮肤肥大细胞增多症的173名儿童。在98名男性和75名女性中,51%患有肥大细胞瘤,47%患有色素性荨麻疹,3名患者患有弥漫性皮肤肥大细胞增多症。在这些病例中,87%在6个月龄之前或6个月龄时首次出现症状。色素性荨麻疹病例中26%出现潮红,肥大细胞瘤病例中29%出现潮红,弥漫性皮肤肥大细胞增多症病例中100%出现潮红。色素性荨麻疹病例中23%出现水疱,肥大细胞瘤病例中31%出现水疱,弥漫性病例中100%出现水疱。色素性荨麻疹病例中59%和肥大细胞瘤病例中49%未出现上述任何一种症状。有3例有阳性家族史。手掌肥大细胞瘤的发现此前仅有一次报道。本文提供了我们病例的图文描述。

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