Department of Dermatology, Yüzüncü Yıl University, Van, Turkey.
Turk J Med Sci. 2014;44(3):504-10. doi: 10.3906/sag-1303-36.
BACKGROUND/AIM: Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells in the skin and sometimes other organs. This study aimed to compare the demographic, clinical, and histopathological findings of cutaneous mastocytosis with onset in children and in adults.
Patients diagnosed with cutaneous mastocytosis in 2 different dermatology clinics between 2007 and 2011 were included in the study. Demographic characteristics of the patients as well as localization and type of the cutaneous lesions, presence of symptoms, Darier's sign, family history, systemic involvement, and histopathological evaluations were retrospectively examined.
Out of the 30 cases of cutaneous mastocytosis, 60% of patients were male (n = 18) and 40% were female (n = 12). Twenty-two patients had childhood-onset mastocytosis (≤15 years) and 8 patients had adult-onset mastocytosis. The onset of the disease occurred before the age of 2 years in all cases of childhood onset. Patients with adult-onset mastocytosis had statistically significantly more systemic involvement than those with childhood-onset mastocytosis (P < 0.05).
Cutaneous mastocytosis is a benign disease in children without systemic involvement and is usually sporadic.
背景/目的：肥大细胞增多症是一组以皮肤和（或）其他器官中肥大细胞异常浸润为特征的异质性疾病。本研究旨在比较儿童和成人发病的皮肤肥大细胞增多症的人口统计学、临床和组织病理学特征。
研究纳入了 2007 年至 2011 年间在 2 家不同皮肤科诊所诊断为皮肤肥大细胞增多症的患者。回顾性检查了患者的人口统计学特征以及皮肤病变的定位和类型、症状、Darier 征、家族史、全身受累和组织病理学评估。
30 例皮肤肥大细胞增多症患者中，60%为男性（n=18），40%为女性（n=12）。22 例为儿童发病（≤15 岁），8 例为成人发病。所有儿童发病的病例均在 2 岁前发病。与儿童发病的肥大细胞增多症相比，成人发病的肥大细胞增多症患者的全身受累情况具有统计学意义（P<0.05）。
儿童发病的皮肤肥大细胞增多症是一种无全身受累且通常为散发性的良性疾病。
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