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新生儿期起病的弥漫性皮肤肥大细胞增多症:一例报告并文献复习

Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature.

作者信息

Koga Hiroshi, Kokubo Takayuki, Akaishi Mutsumi, Iida Koichi, Korematsu Seigo

机构信息

Department of Neonatology, Center for Perinatal Medicine, Oita Prefectural Hospital, Beppu-city, Oita, Japan.

出版信息

Pediatr Dermatol. 2011 Sep-Oct;28(5):542-6. doi: 10.1111/j.1525-1470.2010.01212.x. Epub 2010 Jul 29.

Abstract

Diffuse cutaneous mastocytosis is a rare variant of mast cell disease with widespread erythroderma, which is normally clinically apparent in early infancy. We report the case of a neonate who presented with diffuse erythrodermic rash and bullous lesions. Biopsy specimens showed a dense dermal infiltrate of mast cells. Serum histamine and tryptase levels were elevated. No somatic mutation of the c-kit gene was found. Blistering ceased at 5 months of age, but atopic dermatitis appeared at 6 months and allergic workup revealed a high level of food-specific IgE. Herein, we describe the case and provide the first review of the literature on neonatal onset diffuse cutaneous mastocytosis to clarify the prognosis of this condition.

摘要

弥漫性皮肤肥大细胞增多症是肥大细胞疾病的一种罕见变体,伴有广泛的红皮病,通常在婴儿早期临床上就很明显。我们报告了一例新生儿,其表现为弥漫性红皮病皮疹和大疱性病变。活检标本显示真皮内有密集的肥大细胞浸润。血清组胺和类胰蛋白酶水平升高。未发现c-kit基因的体细胞突变。水疱在5个月大时停止出现,但6个月时出现了特应性皮炎,过敏检查显示食物特异性IgE水平很高。在此,我们描述该病例,并首次对新生儿期发病的弥漫性皮肤肥大细胞增多症的文献进行综述,以阐明这种疾病的预后。

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