Lee Eui Hyung, Kim Mi Ri, Kang Tae Won, Kim Soo-Chan
Department of Dermatology and the Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
Ann Dermatol. 2010 Feb;22(1):77-80. doi: 10.5021/ad.2010.22.1.77. Epub 2010 Feb 28.
We report on a 9-month-old female infant with multiple tense bullae and erosions covering the entire body, including the face, scalp, and trunk. The histopathological examination revealed sub-epidermal bullae with a dense dermal cellular infiltrate. The infiltrate was identified as a collection of mast cells using toluidine blue and Giemsa stains. The direct immunofluorscence was negative. A diagnosis of cutaneous diffuse mastocytosis with generalized bullae was made based on these clinical and histopathological findings. In cases with diffuse cutaneous mastocytosis with generalized bullae, systemic involvement is more frequent and more severe compared to other types of cutaneous mastocytosis. Some lethal outcomes have been reported. This is the first reported case of diffuse cutaneous mastocytosis in the Korean literature.
我们报告了一名9个月大的女婴，其全身包括面部、头皮和躯干出现多个紧张性大疱和糜烂。组织病理学检查显示表皮下大疱，真皮有密集的细胞浸润。使用甲苯胺蓝和吉姆萨染色将浸润物鉴定为肥大细胞聚集。直接免疫荧光检查为阴性。根据这些临床和组织病理学发现，诊断为伴有全身性大疱的皮肤弥漫性肥大细胞增多症。与其他类型的皮肤肥大细胞增多症相比，伴有全身性大疱的皮肤弥漫性肥大细胞增多症的系统性受累更频繁且更严重。已有一些致命结局的报道。这是韩国文献中首次报道的皮肤弥漫性肥大细胞增多症病例。
