Juratli H A, Sárdy M
Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg, Standort Marburg, Baldingerstr. 1, 35043, Marburg, Deutschland.
Klinik für Dermatologie, Venerologie und Dermatoonkologie, Fakultät für Medizin, Semmelweis Universität, Budapest, Ungarn.
Hautarzt. 2019 Apr;70(4):254-259. doi: 10.1007/s00105-019-4377-9.
Linear IgA bullous dermatosis is a rare autoimmune blistering disease that occurs in both children and adults. Strings of pearls, crowns of jewels, rosettes and urticarial plaques can occur on the whole integument with emphasis on the face (particularly perioral area) and genitalia. Pruritus is common and may be severe. The presence of IgA deposits along the basement membrane can usually be identified using direct immunofluorescence (DIF) microscopy. The histological and clinical features of this disorder may mimic those of dermatitis herpetiformis.
线状IgA大疱性皮肤病是一种罕见的自身免疫性大疱性疾病，可发生于儿童和成人。全身皮肤可出现珍珠串、宝石冠、玫瑰花结和荨麻疹样斑块，以面部（尤其是口周区域）和生殖器部位最为明显。瘙痒常见，且可能较为严重。沿基底膜的IgA沉积通常可通过直接免疫荧光（DIF）显微镜检查来识别。该疾病的组织学和临床特征可能与疱疹样皮炎相似。
