Larrègue M, Bressieux J M, Bureau B, Gallet P, Rat J P, Pinalie A, de Giacomoni P
Ann Dermatol Venereol. 1980 May;107(5):389-95.
The observation of pemphigus foliaceus out of endemic zone in a six-year-old child is reported. The rate of anti-ICS antibodies is very high (1/6,400). The direct immunofluorescence shows the common aspect of epidermic network between the cells. Fluorescence basement membrane has been found with monospecific anti-C3 sera, and after remission with the monospecific anti-IgG sera. The clinical, histological and immunological relationship between pemphigus foliaceus in its sporadic and endemic forms and pemphigus erythematosus are discussed. The clinical and immunological regression is obtained by association of prednisone and immunodepressive therapy. After two years of treatment the maintenance dosis is not yet reached. A review of ten previous reported cases of pemphigus foliaceus in children in its sporadic form is presented.
报告了一名6岁儿童在非流行区发生落叶型天疱疮的病例。抗表皮细胞间抗体率非常高（1/6400）。直接免疫荧光显示细胞间表皮网络的常见表现。用单特异性抗C3血清发现荧光基底膜，缓解后用单特异性抗IgG血清也发现荧光基底膜。讨论了散发性和地方性落叶型天疱疮与红斑型天疱疮之间的临床、组织学和免疫学关系。通过泼尼松和免疫抑制疗法联合应用实现了临床和免疫缓解。治疗两年后尚未达到维持剂量。还介绍了此前报道的10例儿童散发性落叶型天疱疮病例的综述。
