Gammon W R, Kowalewski C, Chorzelski T P, Kumar V, Briggaman R A, Beutner E H
Department of Dermatology, University of North Carolina, Chapel Hill 27514.
J Am Acad Dermatol. 1990 Apr;22(4):664-70. doi: 10.1016/0190-9622(90)70094-x.
Bullous pemphigoid and epidermolysis bullosa acquisita may have indistinguishable clinical, histologic, and routine immunohistologic features. In those cases these two diseases can be reliably distinguished in routine diagnostic studies only in seropositive cases by tests on lamina lucida-split skin and in research studies by direct immunoelectron microscopy or, in patients with circulating autoantibodies, by immunoblotting studies. The use of these methods is limited by the expense and unavailability of the methods, the requirement for circulating autoantibodies, or both. We describe a method to distinguish between the two diseases on the basis of findings of direct immunofluorescence of a biopsy specimen after separation through the lamina lucida with 1.0 mol/L sodium chloride. The IgG appeared in the dermal side of the split specimens in epidermolysis bullosa acquisita and predominantly or exclusively in the epidermal side in pemphigoid. The method was found to be relatively simple, inexpensive, applicable to specimens preserved in transport media, and 100% reliable in our group of 22 patients.
大疱性类天疱疮和获得性大疱性表皮松解症可能具有难以区分的临床、组织学和常规免疫组织学特征。在这些病例中,仅在血清阳性病例中,通过对透明层分离皮肤进行检测,以及在研究中通过直接免疫电子显微镜检查,或者在有循环自身抗体的患者中通过免疫印迹研究,这两种疾病才能在常规诊断研究中得到可靠区分。这些方法的使用受到方法费用高、难以获得、需要循环自身抗体或两者兼有的限制。我们描述了一种基于活检标本经1.0 mol/L氯化钠透过透明层分离后直接免疫荧光结果来区分这两种疾病的方法。在获得性大疱性表皮松解症中,IgG出现在分离标本的真皮侧,而在类天疱疮中主要或仅出现在表皮侧。该方法相对简单、便宜,适用于保存在运输介质中的标本,在我们的22例患者组中100%可靠。