关键词: 淀粉样变性, 苔藓状淀粉样变, 瘙痒症, 度普利尤单抗, 治疗

Abstract: 【Abstract】 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.

Key words: Amyloidosis, Lichen amyloidosis, Pruritus, Dupilumab, Therapy