Bystryn J C, Rodriguez J
J Clin Invest. 1978 Feb;61(2):339-48. doi: 10.1172/JCI108944.
12 patients with pemphigus foliaceus, a form of pemphigus with lesions that arise in the intercellular substance in the superficial layers of the epidermis, and 7 patients with pemphigus vulgaris, where lesions are in the deep layers, were studied by immunofluorescence. Circulating antibodies to intercellular antigens (IC antibodies) were found in 11 pemphigus foliaceus and 5 pemphigus vulgaris patients. On direct immunofluorescence of skin lesions 75% (9 of 12), pemphigus foliaceus patients had intercellular deposits of IgG localized solely or predominantly in the superficial epidermal layers, whereas this was not the case in any of the patients with pemphigus vulgaris. Over 70% of the pemphigus foliaceus patients with predominantly superficial IgG deposits lacked in their lesions normal intercellular antigens usually expressed in the deep layers of the epidermis. This was shown by the inability of IC antibodies in autologous or allogeneic sera to bind to intercellular antigens in the lower epidermis of patient's skin, even though the same sera could bind to intercellular antigens in all layers of normal allogeneic skin. Lack of normal intercellular antigens deep in the epidermis may result in circulating IC antibodies binding to the superficial layers, a site which corresponds to, and thus in some patients may account for, the anatomical location of lesions in pemphigus foliaceus.
对12例落叶型天疱疮患者(一种天疱疮,其病损出现在表皮浅层的细胞间质中)和7例寻常型天疱疮患者(病损位于深层)进行了免疫荧光研究。在11例落叶型天疱疮患者和5例寻常型天疱疮患者中发现了针对细胞间抗原的循环抗体(IC抗体)。在皮肤损害的直接免疫荧光检查中,75%(12例中的9例)的落叶型天疱疮患者有IgG的细胞间沉积,仅局限于或主要位于表皮浅层,而寻常型天疱疮患者均未出现这种情况。超过70%的主要有浅层IgG沉积的落叶型天疱疮患者,其损害中缺乏通常在表皮深层表达的正常细胞间抗原。这表现为自体或异体血清中的IC抗体无法与患者皮肤下表皮的细胞间抗原结合,尽管相同的血清能与正常异体皮肤各层的细胞间抗原结合。表皮深层缺乏正常细胞间抗原可能导致循环IC抗体与浅层结合,这个部位与落叶型天疱疮病损的解剖位置相对应,因此在一些患者中可能解释了病损的位置。