Shy S W, Lee W H, Chang Y C, Wu W
Department of Pathology, Lotung Poh Ai Hospital, Yilan, Taiwan, Republic of China.
Pathol Res Pract. 1996 Aug;192(8):869-74; discussion 875-6. doi: 10.1016/S0344-0338(96)80064-1.
Congenital self-healing histiocytosis (CSHH), and especially the solitary variant, is a rare primary skin disorder. We report on a newborn with a congenital solitary ulcerated skin nodule. Extracutaneous involvement was not found. A skin biopsy was performed at the age of 44 days and revealed a dense dermal infiltrate of large histiocytic cells. Immunohistochemical study revealed that the cells of the dermal infiltrate were Langerhans' cells which were strongly positive for S- 100 and negative for lysozyme, leukocyte common antigen and alpha-1-antichymotrypsin. The skin lesion involuted spontaneously over the next month without any treatment. The clinical, histopathological and immunohistochemical results fulfilled the criteria of solitary CSHH. We herein report the first case of CSHH in Taiwan and the twelfth case of solitary CSHH in the world.
先天性自愈性组织细胞增多症（CSHH），尤其是孤立性变体，是一种罕见的原发性皮肤疾病。我们报告了一名患有先天性孤立性溃疡性皮肤结节的新生儿。未发现皮肤外受累情况。在44天时进行了皮肤活检，结果显示真皮内有大量组织细胞密集浸润。免疫组织化学研究表明，真皮浸润细胞为朗格汉斯细胞，S-100呈强阳性，而溶菌酶、白细胞共同抗原和α-1抗糜蛋白酶呈阴性。在接下来的一个月里，皮肤病变未经任何治疗就自行消退了。临床、组织病理学和免疫组织化学结果均符合孤立性CSHH的标准。我们在此报告台湾首例CSHH病例以及世界上第十二例孤立性CSHH病例。
