Cai Sophie Carrie Shan, Tee Shang Ian, Lee Joyce Siong See, Tan Lucinda Siyun
National Skin Centre, Singapore.
Am J Dermatopathol. 2020 Feb;42(2):136-139. doi: 10.1097/DAD.0000000000001550.
We report an unusual case of a 49-year-old woman who presented with persistent papulonodules over bilateral fingers and inframammary region in conjunction with features of connective tissue disease including symmetrical polyarthritis and Raynaud phenomenon. Skin biopsy showed an upper-to-mid dermal proliferation of bland spindled cells with thickened collagen bundles and occasional multinucleated giant cells. Dermal blood vessels were only marginally increased. On immunohistochemistry, both the spindled cells and multinucleated giant cells stained negatively for smooth muscle actin. Some of the spindled cells stained positively with CD68 and CD163, whereas the multinucleated giant cells stained negatively for both stains. Elastic fibers were absent on elastic Van Gieson. The clinical and histopathologic features raise a diagnostic dilemma between fibroblastic rheumatism and multinucleate cell angiohistiocytoma. The patient responded well to cyclosporine and methotrexate therapy, with gradual improvement of the finger nodules.
我们报告了一例罕见病例，一名49岁女性，双侧手指及乳房下区域出现持续性丘疹结节，并伴有结缔组织病特征，包括对称性多关节炎和雷诺现象。皮肤活检显示真皮上部至中部有温和的梭形细胞增生，胶原束增厚，偶见多核巨细胞。真皮血管仅略有增多。免疫组化显示，梭形细胞和多核巨细胞平滑肌肌动蛋白染色均为阴性。部分梭形细胞CD68和CD163染色呈阳性，而多核巨细胞这两种染色均为阴性。弹性Van Gieson染色未见弹性纤维。临床和组织病理学特征在诊断上给成纤维细胞性风湿病和多核细胞血管组织细胞瘤带来了两难困境。该患者对环孢素和甲氨蝶呤治疗反应良好，手指结节逐渐改善。
