Graff-Lonnevig V, Kaaman T
Sachs' Children's Hospital, Stockholm, Sweden.
Acta Paediatr Scand. 1991 Feb;80(2):262-5. doi: 10.1111/j.1651-2227.1991.tb11846.x.
Pemphigus vulgaris is extremely rare in childhood. Only 35 cases of juvenile pemphigus vulgaris have been reported in the literature up until to now. During the pre-steroid era, the outcome of the disease was usually regarded as fatal. Today, the prognosis has improved because of the possibility of early diagnosis by immunofluorescent techniques, determination of antibodies against epidermal intercellular substance and early therapy with systemic steroids. A case of juvenile pemphigus vulgaris with a favourable outcome in a 13-year old girl is described.
寻常型天疱疮在儿童期极为罕见。截至目前,文献中仅报道了35例青少年寻常型天疱疮病例。在类固醇激素出现之前的时代,该病的预后通常被认为是致命的。如今,由于免疫荧光技术可实现早期诊断、能检测抗表皮细胞间物质抗体以及可采用全身性类固醇激素进行早期治疗,预后已有所改善。本文描述了一名13岁女孩患青少年寻常型天疱疮且预后良好的病例。