Akoglu G, Erkin G, Cakir B, Boztepe G, Sahin S, Karaduman A, Atakan N, Akan T, Kolemen F
Department of Dermatology, Haceteppe University Faculty of Medicine, Ankara, Turkey.
J Eur Acad Dermatol Venereol. 2006 Sep;20(8):969-73. doi: 10.1111/j.1468-3083.2006.01696.x.
Mastocytosis is a rare, heterogeneous group of disorder with abnormal increase of mast cells in one or more organ systems.
To evaluate the demographic and clinical features of cutaneous mastocytosis (CM).
Records of 55 patients with cutaneous mastocytosis were retrospectively analysed.
Of the 22 females and 33 males, 80% had urticaria pigmentosa/maculopapular CM and 20% had mastocytoma. Of all cases, 81.8% had first lesions in childhood. The most common presentation was involvement of trunk together with extremities. Thirteen (23.6%) patients had history of bulla; Darier's sign was positive in 34 of 38 patients. Itching was the most common complaint, provocated by hot weather/bath.
Clinical presentations of urticaria pigmentosa/maculopapular CM and mastocytoma are similar regarding gender, age of onset, age of diagnosis, and presence of Darier's sign and history of bulla. In contrast to mastocytoma, urticaria pigmentosa/maculopapular CM lesions were frequently located on trunk together with extremities.
肥大细胞增多症是一组罕见的异质性疾病，其特征为一个或多个器官系统中肥大细胞异常增多。
评估皮肤肥大细胞增多症（CM）的人口统计学和临床特征。
对55例皮肤肥大细胞增多症患者的记录进行回顾性分析。
22例女性和33例男性中，80%患有色素性荨麻疹/斑丘疹性CM，20%患有肥大细胞瘤。所有病例中，81.8%在儿童期出现首发皮损。最常见的表现是躯干和四肢受累。13例（23.6%）患者有大疱病史；38例患者中有34例Darier征阳性。瘙痒是最常见的主诉，由炎热天气/洗澡诱发。
色素性荨麻疹/斑丘疹性CM和肥大细胞瘤在性别、发病年龄、诊断年龄、Darier征的存在以及大疱病史方面的临床表现相似。与肥大细胞瘤不同，色素性荨麻疹/斑丘疹性CM皮损常位于躯干和四肢。
