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目的:分析生酮饮食与减肥手术后发生酮症相关的色素性痒疹的临床特点。方法:收集2018年9月至2020年9月北京大学人民医院皮肤科诊治的酮症相关色素性痒疹患者的临床资料，总结其临床特点、转归及饮食调节的治疗效果。结果:收集酮症相关的色素性痒疹患者6例，其中女5例，均为生酮饮食后发生；男1例，为减肥手术后发生。主要累及部位包括胸、背、腰、腹部，少见累及部位包括眼睑、腋下、肘窝、阴阜。主要皮损形态为网状分布的荨麻疹样红斑、丘疹、色素沉着，少见皮损形态为绿豆至黄豆大小水疱，疱壁易破。5例患者行尿常规分析，4例尿酮体阳性（+ ~ ++++），3例尿蛋白阳性（+）。2例患者行皮损组织病理检查，显示表皮海绵水肿，散在坏死角质形成细胞，可见基底细胞液化，真皮浅层淋巴细胞浸润，红细胞外渗。给予6例患者进食主食的建议，5例1周内基本治愈，1例因希望继续生酮减肥，在仍然限制主食的基础上，加用米诺环素100 mg/d，症状也明显好转。6例患者尿酮体、尿蛋白均在治疗1周内恢复正常。结论:酮症在色素性痒疹的发生中起重要作用。酮症相关的色素性痒疹可单独采用饮食调节治疗，也可采用米诺环素等药物辅助治疗。“,”Objective:To analyze clinical characteristics of ketosis-associated prurigo pigmentosa after ketogenic diet and bariatric surgery.Methods:Clinical data were collected from patients with ketosis-associated prurigo pigmentosa, who were diagnosed and treated in Department of Dermatology, Peking University People′s Hospital from September 2018 to September 2020. The clinical characteristics, sequelae and therapeutic effect of dietary modification were analyzed and summarized.Results:A total of 6 patients with ketosis-associated prurigo pigmentosa were collected, including 5 females who developed prurigo pigmentosa after ketogenic diet, and 1 male who developed prurigo pigmentosa after bariatric surgery. The skin lesions mainly involved the chest, back, waist and abdomen, and rarely involved the eyelids, axillae, elbows and mons pubis. Common skin lesions included urticaria-like erythema, papules and pigmentation arranged in a reticular distribution, and rare skin lesions included mung bean- to soybean-sized blisters, whose walls were liable to break. Among 5 patients undergoing routine urine analysis, 4 were positive (from + to ++++) for ketone bodies in the urine, and 3 were positive for urinary protein (+) . Pathological examination in 2 patients showed epidermal spongiosis, scattered necrotic keratinocytes, basal cell liquefaction, lymphocyte infiltration in the superficial dermis, and erythrocyte extravasation. The 6 patients were advised to eat staple foods. After dietary modification, 5 patients were nearly cured within 1 week; 1 patient, who continued ketogenic diet for weight loss, still received marked improvement after the treatment with minocycline at a dose of 100 mg/d in spite of restriction of carbohydrate intake. The levels of urinary ketone bodies and urinary protein in the 6 patients all returned to normal within 1 week after treatment.Conclusions:Ketosis plays an important role in the occurrence of prurigo pigmentosa. Dietary modification alone or adjuvant medical treatment such as minocycline is effective for the treatment of ketosis-related prurigo pigmentosa.