PDF(Pubmed)
Abstract：
Mastocytosis is a disease of the mast cells caused by an increase in the number of mast cells due to abnormal proliferation. The disease is associated with a mutation in the c-kit gene, which is a key factor in the development of mast cells. Mastocytosis is classified into two main groups, namely, cutaneous and systemic mastocytosis, based on the site of mast cell accumulation. In cutaneous mastocytosis, the cells purely gather in the skin. In contrast, systemic mastocytosis must affect an internal organ, including the bone marrow, lymph nodes, liver, spleen, and/or the gastrointestinal tract with or without skin involvement. Cutaneous mastocytosis has four distinct presentations, including urticaria pigmentosa, cutaneous mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptive perstans listed from most to least common. This case report presents a rare bullous variant of diffuse cutaneous mastocytosis.
摘要：
肥大细胞增多是由肥大细胞数量因异常增殖而增加引起的肥大细胞疾病。这种疾病与c-kit基因突变有关,这是肥大细胞发育的关键因素。肥大细胞增多症分为两大类，即,皮肤和全身性肥大细胞增多症，基于肥大细胞积累的部位.在皮肤肥大细胞增生症中，细胞纯粹聚集在皮肤中。相比之下,系统性肥大细胞增多症必须影响内脏器官，包括骨髓,淋巴结,肝脏,脾,脾和/或有或没有皮肤受累的胃肠道。皮肤肥大细胞增多症有四种不同的表现，包括色素性荨麻疹,皮肤肥大细胞瘤,弥漫性皮肤肥大细胞增多症,从最常见到最不常见的黄斑毛细血管扩张症。该病例报告提出了一种罕见的弥漫性皮肤肥大细胞增多症大疱性变异型。
