Department of Dermatology & Skin Science and Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada.
Exp Dermatol. 2012 Jun;21(6):417-9. doi: 10.1111/j.1600-0625.2012.01475.x. Epub 2012 Apr 16.
Morphea is an autoimmune sclerotic skin disease of unknown pathogenesis. As type 1 interferons (IFN) have been implicated in the pathogenesis of systemic sclerosis, we proposed that type 1 IFN promote localized inflammation and fibrosis in morphea. To investigate the expression of the type 1 IFN-inducible protein myxovirus A (MxA) and the presence of plasmacytoid dendritic cells (pDC) in lesions of morphea, lesional skin of 10 patients with morphea was examined by immunohistochemistry for the presence of the type 1 IFN-inducible protein, myxovirus A (MxA), and the pDC markers, CD123 and BDCA-2, and was compared with lesional skin of cutaneous lupus erythematosus, lichen planus and keloid. Lesional and non-lesional morphea skin was compared. MxA was expressed in the epidermis as well as the reticular dermis and subcutis in morphea. pDCs were abundant around vessels and between fibrous bundles. Non-lesional biopsies demonstrated little or no expression of MxA and pDC markers. Keloid showed minimal expression of MxA and pDC markers. We demonstrate the expression of type 1 IFN-related protein MxA and plasmacytoid DCs in lesional but not in non-lesional biopsies of morphea. These findings suggest a potential role for type 1 interferons in the pathogenesis of morphea.
硬斑病是一种病因不明的自身免疫性硬化性皮肤病。由于 1 型干扰素（IFN）已被认为与系统性硬皮病的发病机制有关，我们假设 1 型 IFN 可促进硬斑病的局部炎症和纤维化。为了研究 1 型 IFN 诱导蛋白 MxA 和浆细胞样树突状细胞（pDC）在硬斑病皮损中的表达情况，我们采用免疫组织化学方法检测了 10 例硬斑病皮损中 1 型 IFN 诱导蛋白 MxA 和 pDC 标志物 CD123 和 BDCA-2 的表达情况，并与皮肤红斑狼疮、扁平苔藓和瘢痕疙瘩皮损进行了比较。比较了硬斑病皮损和非皮损皮肤。MxA 在硬斑病的表皮以及网状真皮和皮下组织中均有表达。pDC 大量存在于血管周围和纤维束之间。非皮损活检显示 MxA 和 pDC 标志物的表达很少或没有。瘢痕疙瘩显示 MxA 和 pDC 标志物的表达最小。我们在硬斑病的皮损而非非皮损活检中证明了 1 型 IFN 相关蛋白 MxA 和浆细胞样树突状细胞的表达。这些发现提示 1 型干扰素可能在硬斑病的发病机制中起作用。
