关键词: Urbach-Wiethe类脂蛋白沉积症

Abstract: Abstract: We report a case of lipoid proteinosis.An 34-year-old Hazakh man, born to unrelated parents, presented with skin eruptions on the body accompanied by hoarseness of unknown origin for more than 20 years. Positive signs of Heart, lung, and abdomen was not found with physical examination. Hoarseness was Obvious. Miliary-sized papules can be seen diffusely on the scalp, face, and neck with sparse hair. Most papules were skincoloured, smooth., and wax. Scarring can be seen. Eyelid thickening, Sparse eyelashes, and beaded wax papulosis in the eyelid margins were also evident. There were symmetric, verrucous hyperplastic, hypertrophic, invasion, pink or color nodule or plaque on elbow and hip. The patient underwent nasopharyngeal laryngoscopy, on account of severe hoarseness, which showed irregular thickening of the vocal cords and epiglottis, and pale yellow hyaline deposits in the larynx, epiglottis and oropharynx. The histopathology of elbow and epiglottis revealed massive deposits of homogeneous, eosinophilic, hyaline-like material in the dermis full-thickness, lamina propria, with perivascular deposits. The material was strongly PAS positive and diastase resistant, with crystal violet negative and congo red negative.