Valeski J E, Kumar V, Beutner E H, Cartone C, Kasprzyk K
Immco Diagnostics, Inc., Buffalo, NY.
Int J Dermatol. 1992 Jan;31(1):37-41. doi: 10.1111/j.1365-4362.1992.tb03518.x.
Patients with bullous pemphigoid and epidermolysis bullosa acquisita may have similar clinical, histologic, and routine immunohistologic features. These two diseases can be distinguished by routine diagnostic studies either on a patient's serum tested by indirect immunofluorescence on salt-split normal skin or by obtaining a fresh perilesional skin biopsy, inducing a split at the lamina lucida, and testing for the site of IgG deposition by direct immunofluorescence. Often the serum studies are negative, while direct immunofluorescent studies yield the characteristic linear IgG staining of the basement membrane zone. To eliminate the need for a repeat biopsy to make a laboratory differential diagnosis, we studied the efficacy of salt-splitting perilesional skin biopsies that had been previously submitted and frozen for routine direct immunofluorescent studies. The biopsies were thawed, salt-split, and processed for direct immunofluorescence. Three epidermolysis bullosa acquisita biopsies and seven bullous pemphigoid biopsies examined demonstrated IgG staining at sites consistent with their respective diagnoses. The IgG appeared in the dermal side of the split biopsies in epidermolysis bullosa acquisita and predominantly, or exclusively, in the epidermal side in bullous pemphigoid. Thus the direct immunofluorescent study of previously frozen and subsequently salt-split skin biopsies may be used for the differential diagnosis of bullous pemphigoid from epidermolysis bullosa acquisita. In most cases, it may eliminate the need for a repeat biopsy.
大疱性类天疱疮和获得性大疱性表皮松解症患者可能具有相似的临床、组织学和常规免疫组织学特征。这两种疾病可通过常规诊断研究加以区分,要么对患者血清进行盐裂正常皮肤间接免疫荧光检测,要么获取新鲜的损害周围皮肤活检标本,诱导透明层分裂,然后通过直接免疫荧光检测IgG沉积部位。血清学研究通常呈阴性,而直接免疫荧光研究则可呈现基底膜带特征性的线性IgG染色。为避免重复活检以进行实验室鉴别诊断,我们研究了先前提交并冷冻用于常规直接免疫荧光研究的损害周围皮肤活检标本盐裂的效果。将活检标本解冻、盐裂并进行直接免疫荧光处理。对3例获得性大疱性表皮松解症活检标本和7例大疱性类天疱疮活检标本进行检查,结果显示IgG染色部位与其各自诊断相符。在获得性大疱性表皮松解症中,IgG出现在盐裂活检标本的真皮侧,而在大疱性类天疱疮中,IgG主要或仅出现在表皮侧。因此,对先前冷冻并随后盐裂的皮肤活检标本进行直接免疫荧光研究可用于大疱性类天疱疮与获得性大疱性表皮松解症的鉴别诊断。在大多数情况下,这可能无需重复活检。