嗜酸细胞增多性血管淋巴样增生伴肾病综合征

来源 :世界核心医学期刊文摘(皮肤病学分册) | 被引量 : 0次 | 上传用户:xienengxian0615
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A 45-year-old woman from Afghanistan presented with a 1year history of multiple itching and stinging lesions on both right and left auricular and periauricular areas. Skin examination revealed about eight superficial erythematous nodules, ranging from 0.2 to 1.2 cm in diameter, on both ears, as well as on the preauricular and retroauricular areas (Fig. 1). Examination of a lesional skin biopsy specimen revealed vascular proliferation and dense dermal infiltration by lymphocytes, eosinophils, and mast cells. The vascular component consisted of thick- and thin- walled blood vessels lined by plump endothelial cells with large nuclei and abundant eosinophilic cytoplasm. No lymphoid follicles were identified (Fig. 2). The patient’ s medical history was remarkable for the gradual development of generalized edema starting 4 months after the appearance of the skin lesions. Laboratory investigation revealed the presence of massive proteinuria (11 g/day; normal,<150 mg/day) and he- maturia (12 red blood cells/high- power field; normal,<1- 2red blood cells/high- power field), in association with hypoproteinemia (3.7 g/dL; normal, 5.5- 8 g/dL and hypercholesterolemia (489 mg/dL; normal, 50- 220 mg/dL). The blood urea nitrogen (14 mg/dL; normal, 5- 23 mg/dL) and serum creatinine (0.8 mg/dL; normal, 0.6- 1.6 mg/dL) levels were within normal limits. A pathologic study of a renal biopsy specimen confirmed the diagnosis of diffuse mesangial proliferative nephropathy (Fig. 3). A 45-year-old woman from Afghanistan presented with a 1year history of multiple itching and stinging lesions on both right and left auricular and periauricular areas. Skin examination revealed about eight superficial erythematous nodules, ranging from 0.2 to 1.2 cm in diameter, on both ears, as well as on the preauricular and retroauricular areas (Fig. 1). Examination of a lesional skin biopsy specimens revealed vascular proliferation and dense dermal infiltration by lymphocytes, eosinophils, and mast cells. The vascular component consisted of thick- and thin- walled blood vessels lined by plump endothelial cells with large nuclei and abundant eosinophilic cytoplasm. No lymphoid follicles were identified (Fig. 2). The patient ’s medical history was remarkable for the gradual development of generalized edema starting 4 months after the appearance of the skin lesions. Laboratory investigation revealed the presence of massive proteinuria (11 g / day; normal, <150 mg / day) and hematuria (12 r ed blood cells / high power field; normal, <1-2 de blood cells / high power field, in association with hypoproteinemia (3.7 g / dL; normal, 5.5-8 g / dL and hypercholesterolemia (489 mg / dL; normal, 5- 23 mg / dL) and serum creatinine (0.8 mg / dL; normal, 0.6-1.6 mg / dL) levels were within normal limits. A pathologic study of a renal biopsy specimen confirmed the diagnosis of diffuse mesangial proliferative nephropathy (Fig. 3).
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