Esterly N B, Furey N L, Kirschner B S, Kretschmer R R, Septon R M
Arch Dermatol. 1977 Jan;113(1):42-6.
The clinical features, laboratory studies, and therapeutic responses of two boys with chronic bullous dermatosis of childhood are described. Direct immunofluorescent preparations of sections from a lesion, skin adjacent to a lesion, and uninvolved skin demonstrated linear deposition of IgA at the dermoepidermal junction in all three biopsy specimens from one patient. Similar preparations from the second child were negative for staining. No circulating antibodies to skin components were detected in either child by means of multiple substrates. Neither child had clinical manifestations nor laboratory findings suggestive of an associated gastrointestinal lesion; therefore, small bowel biopsies were not performed. Immunologic studies failed to demonstrate any of the abnormalities frequently described in dermatitis herpetiformis. Both boys responded dramatically to sulfapyridine therapy.
本文描述了两名患有儿童慢性大疱性皮肤病男孩的临床特征、实验室检查及治疗反应。对一名患者取自皮损处、皮损周边皮肤及未受累皮肤的切片进行直接免疫荧光检查，结果显示在所有这三个活检标本的真皮表皮交界处均有IgA呈线性沉积。取自第二名患儿的类似标本染色为阴性。通过多种底物检测，两名患儿均未检测到针对皮肤成分的循环抗体。两名患儿均无提示存在相关胃肠道病变的临床表现及实验室检查结果；因此，未进行小肠活检。免疫学研究未能证实疱疹样皮炎中常见的任何异常情况。两名男孩对磺胺吡啶治疗均有显著反应。
