作者报道2例类脂蛋白沉积症(Urbach-Wiethe氏病)。例1,白人,女性,23岁,患有皮肤结节病变及声音嘶哑,症状出现于生后6个月及2岁。查体:皮肤显示角化病性丘疹,面部及唇显示黄色丘疹。皮肤干燥,舌增大及粘膜有结节。牙龈组织异常肥大。由于舌麻木及会厌增厚,间接喉镜检查不能进行。试验室检查包括血常规、尿分析均正常。听力测定显示双侧 The authors report two cases of lipoproteinosis (Urbach-Wiethe’s disease). Example 1, white, female, 23 years old, suffering from skin nodules and hoarseness. Symptoms appear 6 months and 2 years after birth. Physical examination: skin showed keratosis papules, face and lip showed yellow papules. Dry skin, enlarged tongue and mucous membranes with nodules. Abnormal gingival tissue hypertrophy. Indirect laryngoscopy can not be performed due to tongue numbness and epiglottis thickening. Laboratory tests, including blood, urinalysis were normal. Audiometry shows both sides