Yamada Tomoko, Suzuki Masayuki, Koike Yumiko, Kida Kinuyo, Murata Satoru, Ishii Norito, Hashimoto Takashi, Ohtsuki Mamitaro
Department of Dermatology, Jichi Medical School, Tochigi, Japan.
Dermatology. 2006;212(4):381-4. doi: 10.1159/000092292.
Some cases of a subepidermal blistering disease associated with autoantibodies to more than two antigens have been reported.
A 52-year-old Japanese woman had pruritic blisters on almost the whole body as well as erosive lesions in the oral cavity and esophagus. A histological finding was subepidermal bullae. Direct immunofluorescence (IF) revealed a linear deposition of IgG, IgM and C3 at the epidermal basement membrane zone (BMZ). Indirect IF using human skin split by 1 M NaCl as a substrate showed IgG antibody reactive with the dermal side. By immunoblot analysis using normal human dermal extract, the 200-kDa and 290-kDa bands were detected. Indirect IF did not show any anti-BMZ antibody activity, when using the skin of the patient with recessive dystrophic epidermolysis bullosa as a substrate.
We regarded our case as epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen. This is the second case in the literature associated with autoantibodies to these two antigens.
有报道称一些大疱性表皮松解症病例与针对两种以上抗原的自身抗体有关。
一名52岁的日本女性全身几乎都出现瘙痒性水疱，口腔和食管有糜烂性病变。组织学检查发现为表皮下水疱。直接免疫荧光（IF）显示在表皮基底膜带（BMZ）有IgG、IgM和C3的线性沉积。以经1 M NaCl分离的人皮肤为底物进行间接免疫荧光显示IgG抗体与真皮侧反应。使用正常人真皮提取物进行免疫印迹分析，检测到200 kDa和290 kDa条带。以隐性营养不良性大疱性表皮松解症患者的皮肤为底物进行间接免疫荧光时，未显示任何抗BMZ抗体活性。
我们将该病例视为获得性大疱性表皮松解症，伴有抗p200类天疱疮抗原自身抗体。这是文献中第二例与这两种抗原自身抗体相关的病例。
