Granulomatous pigmented purpuric dermatitis associated with primary Sjögren's syndrome.

Granulomatous pigmented purpuric dermatitis associated with primary Sjögren's syndrome.
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与原发性干燥综合征相关的肉芽肿性色素性紫癜皮炎。

DOI:
10.2340/00015555-1380
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发表时间:
2013
影响因子:
3.6
通讯作者:
S. Aiba
S. Aiba
中科院分区:
医学3区
文献类型:
--
作者:
Chihiro Wakusawa;T. Fujimura;T. Haga;S. Aiba

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©2013作者。doi: 10.2340/00015555-1380期刊编辑©2013皮肤性病学报。色素性紫癜性皮肤病(GPPD)的肉芽肿变异体在1996年首次被描述(1)。这些报告表明GPPD与自身免疫性疾病之间存在显著关系(2-4)。此外,Kaplan等人(2)最近报道了GPPD的免疫学特征,并描述了肉芽肿组织中大量CD4+细胞的浸润,提示与结节病一样,免疫机制可能与肉芽肿的形成有关。Sjögren综合征(SS)是一种自身免疫性疾病,以泪腺和唾液腺淋巴细胞浸润引起的眼干和口干为特征。SS的各种皮肤表现包括皮肤干燥、免疫炎症如血管炎和高γ -球蛋白血症性紫癜(4)。其中,在极少数情况下,SS伴有肉芽肿性疾病,如结节病(5)。我们在这里描述了一例GPPD与SS相关的病例,并展示了肉芽肿形成细胞的免疫组织化学染色,特别是Foxp3+调节性T细胞。
© 2013 The Authors. doi: 10.2340/00015555-1380 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 Granulomatous variants of pigmented purpuric dermatoses (GPPD) were first described in 1996 (1). These reports suggested significant relationships between GPPD and autoimmune disorders (2–4). In addition, Kaplan et al. (2) recently reported the immunological profiles of GPPD and described the infiltration of significant numbers of CD4+ cells in granulomatous tissues, suggesting that, like sarcoidosis, immunological mechanisms might be associated with the formation of granuloma. Sjögren’s syndrome (SS) is an autoimmune disorder characterized by dry eyes and dry mouth due to lympho­ cytic infiltrates in the lacrimal and salivary glands. The various cutaneous manifestations of SS include dry skin, immunological inflammatory conditions such as vasculitis, and hypergammaglobulinemic purpura (4). Among them, in rare cases, SS is associated with granulomatous disorders, such as sarcoidosis (5). We describe here a case of GPPD associated with SS and demonstrate immunohistochemical staining for granuloma­forming cells, focusing especially on Foxp3+ regulatory T cells.