Sillevis Smitt J H, Leusen J H, Stas H G, Teeuw A H, Weening R S
Department of Dermatology, University of Amsterdam, Netherlands.
Arch Dis Child. 1997 Aug;77(2):150-2. doi: 10.1136/adc.77.2.150.
A 12 year old boy suffering from p67-phox deficient chronic granulomatous disease presented with a bullous skin disease and a lung infection with paecilomyces species. The histopathology of a bullous lesion showed subepidermal blister formation and microabcesses containing eosinophils in the dermal papillae. By direct immunofluorescence, linear staining of IgA at the dermal-epidermal junction was detected which confirmed the clinical diagnosis of chronic bullous disease of childhood (linear IgA dermatosis).
一名患有p67 - phox缺陷型慢性肉芽肿病的12岁男孩出现大疱性皮肤病和拟青霉菌肺部感染。一个大疱性病变的组织病理学显示表皮下疱形成,真皮乳头中有含嗜酸性粒细胞的微脓肿。通过直接免疫荧光法,在真皮 - 表皮交界处检测到IgA呈线性染色,这证实了儿童慢性大疱性疾病(线性IgA皮肤病)的临床诊断。