Ben-Amitai Dan, Metzker Aryeh, Cohen Herman A
Schneider Children's Medical Center of Israel, Petah Tiqva, Israel.
Isr Med Assoc J. 2005 May;7(5):320-2.
Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells in the skin and, sometimes, other organs. Some patients may experience symptoms related to mast cell mediator release.
To analyze the clinical features of cutaneous mastocytosis in a large series of children.
We conducted a file review of all children clinically diagnosed with cutaneous mastocytosis in our department over the last 20 years. We evaluated gender, age at onset, character and distribution of the lesions, associated symptoms, and course of the disease.
Altogether, 180 patients with cutaneous mastocytosis were identified. The male to female ratio was 1.5:1. About one-third of patients had a mastocytoma, which was present at birth in over 40% and appeared during the first year of life in most of the remainder. Urticaria pigmentosa was noted in 65% of the patients, presenting at birth in 20% and during the first year in most of the remainder. The majority of lesions was distributed over the trunk and limbs. Different kinds of associated symptoms were noted. Prognosis in general was good. Only 11% of the cases, all urticaria pigmentosa, were familial.
Most cases of pediatric mastocytosis are sporadic and appear during the first 2 years of life, especially on the trunk. Urticaria pigmentosa is the most frequent variant. The prognosis of pediatric mastocytosis, in general, is good.
肥大细胞增多症是一组异质性疾病，其特征为肥大细胞在皮肤以及有时在其他器官中异常浸润。一些患者可能会出现与肥大细胞介质释放相关的症状。
分析大量儿童皮肤肥大细胞增多症的临床特征。
我们对过去20年在我科临床诊断为皮肤肥大细胞增多症的所有儿童病历进行了回顾。我们评估了性别、发病年龄、皮损的特征和分布、相关症状以及病程。
共确定了180例皮肤肥大细胞增多症患者。男女比例为1.5:1。约三分之一的患者患有肥大细胞瘤，其中40%以上在出生时即存在，其余大部分在出生后第一年出现。65%的患者有色素性荨麻疹，20%在出生时出现，其余大部分在出生后第一年出现。大多数皮损分布于躯干和四肢。观察到不同类型的相关症状。总体预后良好。仅11%的病例（均为色素性荨麻疹）为家族性。
大多数儿童肥大细胞增多症病例为散发性，在出生后头2年内出现，尤其是在躯干。色素性荨麻疹是最常见的类型。儿童肥大细胞增多症总体预后良好。
