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抗BP180、BP230和层粘连蛋白5自身抗体阳性乌干达患者的瘢痕形成性自身免疫性大疱病

世界核心医学期刊文摘（皮肤病学分册） 2006年第4期2卷 13-14页

作者： mulyowa g.k. Jaeger g. Sitaru C. E. Schmidt 任建文 Department of Dermatology University of Wü rzburg Josef-Schneider-Str 2 97080 Wü rzburg Germany Dr.

We report on a 24-year-old, male Ugandan patient with a 2-week history of itchy papules, vesicles, erosions, and crusts distributed on the entire body, accompanied by minor erosions on the palate, tongue, and lower lip. Conjunctivae and genital mucosa were not involved. Circulating Igg and IgA autoantibodies were found against recombinant full-length BP180, BP180 4575, and the C-terminus of BP230. In addition, Igg reactivity was observed against the 16th noncollagenous region of the BP180 ectodomain, the cell-derived soluble ectodomain of BP180 (linear IgA disease antigen 1), and the α 3 and γ 2 chains of laminin 5. No reactivity was detected with type VII collagen, α 6β 4 integrin, and the p200 protein. Oral prednisolone and dapsone led to clearance of lesions that mostly healed with scarring and milia formation. Here, we describe a scarring mucocutaneous variant of an autoimmune blistering skin disorder that extends the current clinical and immunopathologic spectrum of this group of diseases.

关键词：大疱层粘连蛋白 BP180 BP230 自身抗体瘢痕形成粟丘疹 Igg 免疫病理眼结膜

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维普期刊数据库

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乌干达自身免疫性表皮下水疱性疾病:自身抗体类别和年龄的相关性

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世界核心医学期刊文摘（皮肤病学分册） 2006年第12期2卷 24-页

作者： mulyowa g.k.Jaeger g. kabakyenga J. E. Schmidt 董平 Department of Dermatology University of Würzburg Josef-Schneider-Str. 2 97080 Würzburg Germany Dr.

Background: No data are available on the incidence and immunoreactivity of autoimmune subepidermal blistering skin diseases in East Africa. Methods: 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 1M NaCl-split human skin and by Western blotting of recombinant and cell-derived forms of BP180, BP230, and type VII collagen. Results: Twenty two patients with autoimmune subepidermal blistering skin disorders were identified, including nine with bullous pemphigoid 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). Conclusions: 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.

关键词：表皮皮肤类天疱疮大疱性皮肤病水疱自身免疫性自身抗体乌干达性疾病

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维普期刊数据库

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