Mulyowa Grace K, Jaeger Gerold, Kabakyenga Jerome, Bröcker Eva-B, Zillikens Detlef, Schmidt Enno
Department of Dermatology, University of Würzburg, Würzburg, Germany.
Int J Dermatol. 2006 Sep;45(9):1047-52. doi: 10.1111/j.1365-4632.2006.02750.x.
No data are available on the incidence and immunoreactivity of autoimmune subepidermal blistering skin diseases in East Africa.
All patients with frank blisters/erosions on the skin and/or mucous membranes that attended the Department of Dermatology at Mbarara University, Uganda, from May 2000 to June 2002, were investigated. The diagnosis was based on the clinical presentation and on the presence of circulating autoantibodies detected by indirect immunofluorescence microscopy on 1 m NaCl-split human skin and by Western blotting of recombinant and cell-derived forms of BP180, BP230, and type VII collagen.
Twenty-two patients with autoimmune subepidermal blistering skin disorders were identified, including nine with bullous pemphigoid (41%), four with linear immunoglobulin A (IgA) disease (18%), three with mucous membrane pemphigoid (14%), two with linear IgG/IgA bullous dermatosis (9%), and one each with cicatricial pemphigoid and epidermolysis bullosa acquisita (5%). In addition, two patients with immunoreactivity to both the epidermal and dermal side of salt-split skin by indirect immunofluorescence microscopy, who were unreactive to type VII collagen, were provisionally diagnosed as "mixed pemphigoid" (9%). In patients with subepidermal blistering diseases, IgG reactivity correlated significantly with old age, whereas younger patients preferentially developed IgA autoantibodies (P = 0.024).
The age of patients with autoimmune subepidermal blistering diseases appears to influence the immunoglobulin class of autoantibodies. The high frequency of IgA autoantibodies in Ugandan patients may be explained by the age distribution of the Ugandan population.
关于东非自身免疫性表皮下大疱性皮肤病的发病率和免疫反应性尚无数据。
对2000年5月至2002年6月期间到乌干达姆巴拉拉大学皮肤科就诊的所有有明显皮肤和/或黏膜水疱/糜烂的患者进行了调查。诊断基于临床表现以及通过间接免疫荧光显微镜在1 m NaCl分离的人皮肤上检测到的循环自身抗体,以及对重组和细胞来源形式的BP180、BP230和VII型胶原进行的蛋白质印迹分析。
确定了22例自身免疫性表皮下大疱性皮肤病患者,其中9例为大疱性类天疱疮(41%),4例为线状免疫球蛋白A(IgA)病(18%),3例为黏膜类天疱疮(14%),2例为线状IgG/IgA大疱性皮肤病(9%),瘢痕性类天疱疮和获得性大疱性表皮松解症各1例(5%)。此外,2例通过间接免疫荧光显微镜对盐裂皮肤的表皮和真皮侧均有免疫反应但对VII型胶原无反应的患者被初步诊断为“混合性类天疱疮”(9%)。在表皮下大疱性疾病患者中,IgG反应性与老年显著相关,而年轻患者则更易产生IgA自身抗体(P = 0.024)。
自身免疫性表皮下大疱性疾病患者的年龄似乎会影响自身抗体的免疫球蛋白类别。乌干达患者中IgA自身抗体的高频率可能由乌干达人群的年龄分布来解释。