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[节段性黄斑丘疹性皮肤肥大细胞增多症]

[Metameric macular and papular skin mastocytosis].

作者信息

Maciejewski M, Debarre J-M, Georgin-Lavialle S, Kettani S, Olschwang S, Guérin-Moreau M, Le Corre Y, Martin L

机构信息

Service de dermatologie, CHU d'Angers, 4, rue Larrey, 49933 Angers cedex 9, France.

Cabinet de dermatologie, 11, rue Georges-Clemenceau, 49300 Cholet, France.

出版信息

Ann Dermatol Venereol. 2017 Mar;144(3):208-211. doi: 10.1016/j.annder.2016.09.041. Epub 2016 Oct 21.

Abstract

BACKGROUND

Mastocytosis is characterised by the presence of abnormal quantities of mastocytes in one or more organs. Although it occurs in systemic forms of mastocytosis, isolated skin involvement is the predominant presentation, particularly in children, in the form of more or less extensive though non-systematic lesions. Herein, we report a case of maculopapular cutaneous mastocytosis that is unusual in terms of its metameric topography.

PATIENTS AND METHODS

A 16-year-old youth presented with an erythematous maculopapular rash of 18 months' duration and involving pruritic inflammatory episodes strictly localised in segment T8 to the left. The skin biopsy showed a significant increase in the number of dermal mastocytes (CD117+). No KIT mutations were found in the skin lesions nor in the unimpaired skin of the opposite side. Further investigations ruled out systemic mastocytis.

DISCUSSION

Herein, we report a case of cutaneous mastocytosis that is unusual in terms of its metameric disposition. There have been only two previous reports of segmental cutaneous mastocytis. The two pathological hypotheses involved precessional dermatitis that renders the skin surface susceptible to homing, and somatic mosaicism (type 1) with local mastocyte proliferation.

摘要

背景

肥大细胞增多症的特征是一个或多个器官中存在数量异常的肥大细胞。尽管它以系统性肥大细胞增多症的形式出现,但孤立的皮肤受累是主要表现形式,尤其是在儿童中,表现为或多或少广泛但非系统性的病变。在此,我们报告一例斑丘疹性皮肤肥大细胞增多症,其节段性分布情况较为罕见。

患者和方法

一名16岁青年,出现持续18个月的红斑丘疹性皮疹,伴有瘙痒性炎症发作,严格局限于左侧T8节段。皮肤活检显示真皮肥大细胞(CD117+)数量显著增加。在皮肤病变处及对侧未受累皮肤中均未发现KIT突变。进一步检查排除了系统性肥大细胞增多症。

讨论

在此,我们报告一例皮肤肥大细胞增多症,其节段性分布情况较为罕见。此前仅有两篇关于节段性皮肤肥大细胞增多症的报道。两种病理假说涉及使皮肤表面易于归巢的移行性皮炎,以及伴有局部肥大细胞增殖的体细胞镶嵌现象(1型)。

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