Bittencourt Maraya de Jesus Semblano, Moure Emanuella Rosyane Duarte, Pies Olga Ten Caten, Mendes Alena Darwich, Deprá Monique Morales, Mello Anna Luiza Piqueira de
Universidade Federal do Pará, Belém, PA, Brazil.
Centro Universitário do Estado do Pará, Belém, PA, Brazil.
An Bras Dermatol. 2015 Jan-Feb;90(1):114-6. doi: 10.1590/abd1806-4841.20153011.
Netherton syndrome is a rare autosomal recessive disease characterized by erythroderma, ichthyosis linearis circumflexa, atopy, failure to thrive and a specific hair shaft abnormality called trichorrhexis invaginata or bamboo hair, considered pathognomonic. We report the case of a 4-year-old boy with erythroderma since birth, growth deficit and chronic diarrhea. Trichoscopy was used to visualize typical bamboo and "golf tee" hair and of key importance to diagnose Netherton syndrome. We suggest the use of this procedure in all children diagnosed with erythroderma.
Netherton综合征是一种罕见的常染色体隐性疾病,其特征为红皮病、匐行性线状鱼鳞病、特应性、生长发育迟缓以及一种称为套叠性脆发症或竹节发的特殊毛干异常,被认为具有诊断意义。我们报告了一名4岁男孩的病例,该男孩自出生以来就患有红皮病、生长发育迟缓及慢性腹泻。通过皮肤镜观察到了典型的竹节发和“高尔夫球杆”样毛发,这对诊断Netherton综合征至关重要。我们建议对所有诊断为红皮病的儿童采用这一检查方法。
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