度普利尤单抗治疗泛发性苔藓状淀粉样变1例国内首报

A case of generalized lichen amyloidosis treated with dupilumab:the first case reported in China

国内报道首例度普利尤单抗治疗泛发性苔藓状皮肤淀粉样变。患者男,70岁,躯干、四肢广泛丘疹伴瘙痒23年。皮肤科检查:躯干、右胫前、双上臂外侧见弥漫性粟粒至绿豆大小褐色半球形丘疹,质硬。血常规嗜酸性粒细胞、血清IgE未见异常。下肢皮损组织病理:表皮角化过度,真皮乳头可见均一红染的团块状物质。免疫组化刚果红染色阳性。瘙痒数字量表评分10分。诊断:泛发性苔藓状皮肤淀粉样变。治疗:皮下注射度普利尤单抗,首次600 mg,之后每2周注射300 mg。治疗第2周时,瘙痒即明显缓解,14周时皮损开始明显消退,18周时前胸、腹部皮疹基本消退,腰背部、四肢皮疹明显消退。未见明显不良反应。

To report a case of generalized lichen amyloidosis successfully treated with dupilumab in China.A 70-year-old male patient presented with extensive itchy papules on the trunk and extremities for 23 years.Skin examination revealed diffuse millet-to mung bean-sized hemispherical brown plaques on the trunk,right anterior shank,and extensor aspect of both upper arms,with a hard texture on palpation.No abnormalities were observed in the blood eosinophil count or serum IgE level.Histopathological examination of the skin lesion on the lower limb showed epidermal hyperkeratosis and homogeneous red-stained lumpy materials in the papillary dermis.Immunohistochemical study showed positive staining with Congo red.The pruritus numerical rating scale score was 10 points.The diagnosis of generalized lichen amyloidosis was confirmed.The patient received subcutaneous injection of dupilumab at an initial dose of 600 mg,followed by an every-2-week regimen at a dose of 300 mg.At week 2 after the start of treatment,pruritus was markedly relieved;at week 14,the skin lesions began to subside markedly;at week 18,the skin lesions on the chest and abdomen nearly completely subsided,and lesions on the lower back and limbs markedly regressed.No obvious adverse reactions were observed.