Cordoliani F, Rybojad M, Verola O, Dallot A, Flageul B, Didierjean L, Drouault Y, Morel P
Service de Dermatologie, Hôpital Saint-Louis, Paris.
Ann Dermatol Venereol. 1995;122(10):671-4.
Intraepidermal IgA pustulosis is a vesiculopustular dermatosis defined by pemphigus type intercellular deposit exclusively of IgA. It is a member of the pemphigus group and may be related to neurtrophilic dermatoses.
A child had vesiculopustular lesions of the limbs since the age of 11 years. Biopsy showed the subcorneal intraepidermal nature of the pustules and exclusive IgA deposit throughout the epiderma. Indirect immunofluorescence and protein immunoelectrophoreses were negative. Immunotransfer to beef tongue extract evidenced a 150-160 kDa band corresponding to IgA and IgG desmoglein. Treatment with general corticosteroids followed by pristinamycin was successful.
This is the first case report showing evidence of antiepidermal antibodies directed against the superficial pemphigus antigen.
表皮内IgA脓疱病是一种水疱脓疱性皮肤病，其定义为仅IgA在天疱疮样细胞间沉积。它是天疱疮组的一员，可能与中性粒细胞性皮肤病有关。
一名儿童自11岁起四肢出现水疱脓疱性损害。活检显示脓疱位于角层下表皮内，整个表皮均有IgA沉积。间接免疫荧光和蛋白免疫电泳均为阴性。向牛舌提取物进行免疫转移显示一条150 - 160 kDa的条带，对应于IgA和IgG桥粒芯糖蛋白。先用全身性皮质类固醇治疗，后用 pristinamycin治疗成功。
这是首例显示针对浅表性天疱疮抗原的抗表皮抗体证据的病例报告。
