Konishi Asako, Fukuoka Miyuki, Nishimura Youichi
Department of Dermatology, Fukui Red Cross Hospital, 2-4-1 Tukimi, Fukui, 918-8055, Japan.
J Dermatol. 2007 Jun;34(6):394-6. doi: 10.1111/j.1346-8138.2007.00296.x.
A 69-year-old woman presented with a 2-year history of an eczematous lesion covering the genital area. Histopathological examination showed deposits of amorphous, eosinophilic material and an infiltrate of plasma cells through the entire dermis into the subcutaneous fatty tissue. Congo red-stained deposits showed apple-green birefringence with polarizing microscopy. On immunohistochemistry, the deposited material was positively stained with anti-lambda light chain antibodies but not with anti-lambda light chain. A diagnosis of primary localized cutaneous amyloidosis (PLCA) was made, and the patient was also diagnosed as having Sjögren's syndrome (SjS) based on clinical and laboratory findings. The lesion of PLCA has spontaneously regressed over a period of 18 months. We report a unique case of PLCA and SjS that clinically demonstrated genital eczematous features and spontaneous involution, and we also describe a possible association between PLCA and SjS.
一名69岁女性，其生殖器区域出现湿疹样病变已有2年病史。组织病理学检查显示，整个真皮层至皮下脂肪组织有非晶质嗜酸性物质沉积及浆细胞浸润。刚果红染色沉积物在偏振显微镜下呈现苹果绿双折射。免疫组织化学检查显示，沉积物质用抗λ轻链抗体呈阳性染色，而用抗κ轻链抗体则未染色。诊断为原发性局限性皮肤淀粉样变（PLCA），基于临床和实验室检查结果，该患者还被诊断为干燥综合征（SjS）。PLCA病变在18个月内自行消退。我们报告了一例独特的PLCA合并SjS病例，该病例临床上表现为生殖器湿疹样特征且自行消退，我们还描述了PLCA与SjS之间可能存在的关联。
