摘要患者男,55岁.临床表现为四肢、躯干紧张性大疱,组织学表现为表皮下水疱,真皮浅层有淋巴细胞、嗜酸性粒细胞等浸润,初步诊断为大疱性类天疱疮(BP).在血清学分析中,发现该患者血清中自身抗体与盐裂皮肤真皮侧结合,且酶联免疫吸附法未检测到BP180、BP230、Ⅶ型胶原抗体,从而排除了大疱性类天疱疮、获得性大疱性表皮松解症的诊断.通过免疫印迹、免疫沉淀检测,发现该患者血清存在与真皮侧200 000蛋白结合的抗体,综合这些特点,诊断该患者为抗P200类天疱疮,即抗层黏连蛋白γ1类天疱疮.

abstractsA 55-year-old male patient presented with tense bullae on the extremities and trunk.Histological examination revealed subepidermal vesicles and superficial dermal infiltration of eosinophils and lymphocytes.The patient was primarily diagnosed with bullous pemphigoid.However,serum autoantibodies of the patient bound to the dermal side of salt-split skin,and no serum antibodies against BP180,BP230 or type Ⅶ collagen were detected by enzyme-linked immunosorbent assay.Hence,the diagnoses of bullous pemphigoid and epidermolysis bullosa acquisita were excluded.As Western blot and immunoprecipitation analysis showed,there existed antibodies capable of binding to a dermal antigen with a relative molecular mass of 200 000 in the serum of the patient.Based on the above findings,the patient was diagnosed as anti-laminin γ1 (p200) pemphigoid.