皮肤窦性组织细胞增生症

A case of cutaneous sinus histiocytosis

报告1例皮肤窦性组织细胞增生症(CSH)。患者女,48岁,仅表现为左面颊部有一斑块。皮损的组织病理特征为真皮及皮下有以组织细胞和淋巴细胞为主的浸润,可见吞噬现象。免疫组化染色组织细胞S-100蛋白和CD68阳性,CD1a标记阴性。阿维A试验性治疗有效。

Sinus histiocytosis with massive lymphadenopathy （SHML） （Rosai-Dorfman syndrome）, is a rare, benign, generally self-limited histiocytic proliferative disorder that is characterized by painless lymphadenopathy, fever, neutrophilia, an elevated erythmcyte sedimentation rate, and polyclonal hypergammaglobulinemia. It affects mainly children and young adults. Although the lymph nodes are the most common sites of involvement, extra nodal infiltrates may arise in the orbits, skin, upper respiratory tract, and bone, as well as other organs. Approximately 10% of patients have had cutaneous infiltrates, but very few of them have had lesions limited to the skin（cutaneous sinus histocytosis, CSH）. Here a 46-year-old female patient suffering from CSH is reported. As the purely asymptomatic cutaneous lesions on her cheek were the only clinical manifestation, the disease had taken almost half a year to reach the real diagnosis. Histologically, the lesions were marked by characteristic variable numbers of pale-staining histocytes with emperipolesis overshadowed by extensive lymphoplasmacytie infiltrates in the background. By immunohistochemical analysis, the characteristic histiocytes were positive for S-100 protein and CD68 and negative for CDla. Treatment with acitretin seemed effective in this case.