Lapiere J C, Guitart J, Ettlin D A, Chen D M, Amagai M, Chan L S
Departments of Dermatology, Northwestern University Medical School, Chicago, IL, USA.
Br J Dermatol. 1998 Nov;139(5):851-4. doi: 10.1046/j.1365-2133.1998.02512.x.
Pemphigus vulgaris (PV) is an immune-mediated blistering skin disease characterized by acantholysis of the suprabasal epidermis and by IgG autoantibodies targeting a desmosomal component, desmoglein 3. IgG alone has been demonstrated to induce acantholysis in some experimental conditions. The role of the complement system in blister formation in PV remains controversial. We describe four consecutive patients with new-onset PV. The acantholytic process occurred in the lower epidermis and colocalized with deposition of complement C3 and the membrane attack complex C5b-9. The colocalization of complement deposition with the acantholytic process in the lower epidermis supports a role for the complement system in blister formation in PV.
寻常型天疱疮（PV）是一种免疫介导的水疱性皮肤病，其特征为基底上层表皮棘层松解，以及靶向桥粒成分桥粒芯糖蛋白3的IgG自身抗体。在某些实验条件下，已证实单独的IgG可诱导棘层松解。补体系统在PV水疱形成中的作用仍存在争议。我们描述了4例新发PV的连续病例。棘层松解过程发生在表皮下层，并与补体C3和膜攻击复合物C5b-9的沉积共定位。补体沉积与表皮下层棘层松解过程的共定位支持补体系统在PV水疱形成中发挥作用。
