Trüeb R M, Bruckner-Tuderman L, Wyss M, Widmer M, Wüthrich B, Burg G
Department of Dermatology, University Hospital of Zürich, Switzerland.
Br J Dermatol. 1995 Apr;132(4):621-5. doi: 10.1111/j.1365-2133.1995.tb08722.x.
We report a 2-year-old girl with a combination of the ectodermal dysplasia, ectrodactyly, cleft lip/palate (EEC) syndrome, distinctive hair abnormalities, scalp dermatitis and atopic disease. To our knowledge, this is the first report of scalp dermatitis in the EEC syndrome. Distinctive structural hair abnormalities shared by the syndromes of ectodermal dysplasia and clefting are helicotrichia, pili torti et canaliculi, and cuticle defects, all of which were observed in the present case. A high incidence of atopic disease in certain subsets of ectodermal dysplasia has been reported. In our patient this manifested as food-induced exacerbation of atopic dermatitis, associated with positive prick tests and significant levels of circulating specific IgE.
我们报告一名2岁女童，患有外胚层发育不良、缺指（趾）畸形、唇腭裂（EEC）综合征，并伴有独特的毛发异常、头皮皮炎和特应性疾病。据我们所知，这是EEC综合征中头皮皮炎的首例报告。外胚层发育不良和腭裂综合征共有的独特结构性毛发异常包括螺旋发、扭曲发及管腔发和角质层缺陷，所有这些在本例中均有观察到。据报道，在某些外胚层发育不良亚组中特应性疾病的发病率较高。在我们的患者中，这表现为食物诱发的特应性皮炎加重，伴有阳性点刺试验和显著水平的循环特异性IgE。
