183例儿童皮肤型肥大细胞增生症临床及预后分析  

Clinical and prognostic analysis of 183 cases of pediatric cutaneous mastocytosis

作　　者：孙娟[1] 张立新[1] 张霞[1] 马琳[1] 

机构地区：[1]首都医科大学附属北京儿童医院皮肤科,北京100045

出　　处：《中华皮肤科杂志》2015年第8期539-541,共3页Chinese Journal of Dermatology

摘　　要：目的：探讨儿童皮肤型肥大细胞增生症的临床特征及预后。方法回顾分析183例儿童皮肤型肥大细胞增生症患者的临床资料，并随访部分患者。结果183例患者中，色素性荨麻疹136例（74.3%），肥大细胞瘤43例（23.5%），弥漫性肥大细胞增生症4例（2.2%）；生后2岁内发病者179例（97.8%）。43例肥大细胞瘤患者中出生即发病者21例（48.8%），出生后至6个月发病者17例（39.5%），136例色素性荨麻疹患者中出生即发病者35例（25.7%），出生至6个月发病者78例（57.3%）。伴随症状记录详细的33例患者中，10例出现伴随症状，其中9例为潮红发作。对45例患者随访3~6年（平均4年），色素性荨麻疹患者1例于11岁时皮损完全消退，18例皮损部分消退；肥大细胞瘤患者1例皮损于8岁时完全消退，7例行皮肤活检后1年内皮损消退。口服抗组胺药可控制患者潮红、风团及水疱等症状；口服糖皮质激素可有效控制弥漫性肥大细胞增生症患儿反复发生的泛发水疱、大疱。结论儿童皮肤型肥大细胞增生症以色素性荨麻疹最常见，其次为肥大细胞瘤。肥大细胞瘤多于出生即发病，而色素性荨麻疹以出生后至6个月发病多见。口服抗组胺药可控制介质释放相关症状。严重的弥漫性肥大细胞增生症患者可口服糖皮质激素控制症状。Objective To characterize clinical features and prognosis of pediatric cutaneous mastocytosis. Methods Clinical data on 183 cases of pediatric cutaneous mastocytosis were collected and retrospectively analyzed. Some patients were followed up. Results Of the 183 patients, 136 （74.3%）had urticaria pigmentosa, 43 （23.5%） mastocytoma, 4 （2.2%）diffuse mastocytosis. The first attack of mastocytosis occurred at birth in 21 （48.8%）patients with mastocytoma and 35（25.7%）patients with urticaria pigmentosa, within 6 months after birth in 17（39.5%）patients with mastocytoma and 78（57.3%）patients with urticaria pigmentosa, and within 2 years after birth in 179（97.8%）out of the 183 patients. Of 33 patients with detailed description of symptoms, 10 had concomitant symptoms, which were flushing in 9 patients. Forty-five patients were followed up for 3 - 6 years （average, 4 years）. The follow-up showed that skin lesions completely regressed in 1 patient with urticaria pigmentosa at 11 years of age, partially regressed in 18 patients. Lesions regressed completely at 8 years of age in 1 patient with mastocytoma, and subsided within 1 year after skin biopsy in 7 patients. Oral antihistamines could control the symptoms of mastocytosis, such as flushing, whealing and blistering, and oral glucocorticoids could effectively control the recurrence of generalized blisters and bullae in patients with diffuse mastocytosis. Conclusions Urticaria pigmentosa appears to be the most common type of cutaneous mastocytosis in children, followed by mastocytoma. Mastocytoma occurs most frequently at birth, while urticaria pigmentosa within 6 months after birth. Oral antihistamines may control inflammatory mediator-related symptoms. Serious diffuse mastocytosis may be controlled by systemic glucocorticoids.

关 键 词：肥大细胞增生症 皮肤 色素性荨麻疹 肥大细胞瘤 皮肤 治疗结果 随访研究 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]