Cauza Karla, Hinterhuber Gabriele, Sterniczky Barbara, Brugger Karin, Pieczkowski Friederike, Karlhofer Franz, Wolff Klaus, Foedinger Dagmar
Division of General Dermatology, Department of Dermatology, University of Vienna, School of Medicine, Austria.
J Am Acad Dermatol. 2004 Aug;51(2 Suppl):S112-7. doi: 10.1016/j.jaad.2004.01.059.
Linear IgA dermatosis is a rare autoimmune bullous skin disease with subepidermal blister formation and linear IgA deposits along the basement membrane zone. We describe two female patients showing erythematous annular plaques with scaling at the margin, strictly localized to the palms in one patient, and also found on the soles and buttocks in the second patient. Histology showed numerous neutrophils in the dermis with an admixture of eosinophils, some subepidermal clefting, and occasional papillary microabscesses. Direct immunofluorescence and immunoelectron microscopy revealed in vivo IgA deposition along the basement membrane zone. One patient cleared after treatment with dapsone. The second patient did not respond to dapsone alone and various immunosuppressive treatment regimens. Considerable improvement was achieved with intravenous immunoglobulin therapy combined with corticosteroid and dapsone.
线状IgA大疱性皮病是一种罕见的自身免疫性大疱性皮肤病，有表皮下疱形成，且沿基底膜带有线状IgA沉积。我们描述了两名女性患者，她们表现为边缘有鳞屑的红斑环状斑块，其中一名患者仅局限于手掌，另一名患者在足底和臀部也有。组织学显示真皮内有大量中性粒细胞，并伴有嗜酸性粒细胞，有一些表皮下裂隙，偶尔有乳头微脓肿。直接免疫荧光和免疫电子显微镜检查显示基底膜带有IgA沉积。一名患者经氨苯砜治疗后痊愈。第二名患者单独使用氨苯砜及各种免疫抑制治疗方案均无反应。静脉注射免疫球蛋白联合皮质类固醇和氨苯砜治疗取得了显著改善。
