Kettelhut B V, Metcalfe D D
Division of Allergy and Clinical Immunology, Children's Hospital Medical Center, Cincinnati, Ohio.
J Invest Dermatol. 1991 Mar;96(3):15S-18S.
The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.
大约55%的肥大细胞增多症病例在出生至2岁之间发病；另有10%在15岁之前患病。这些年龄组的肥大细胞增多症在许多方面与成年期发病的肥大细胞增多症不同。儿童期发病的肥大细胞增多症的典型表现为皮肤表现：单发肥大细胞瘤、色素性荨麻疹，或较少见的弥漫性皮肤肥大细胞增多症。特别是在婴儿中，可能会出现大疱性皮疹。婴儿和儿童的肥大细胞增多症可能累及包括骨髓和胃肠道在内的内部器官，尽管这种表现在儿童中似乎比在成人中少见。儿童期发病的肥大细胞增多症患者血浆组胺水平可能升高。治疗通常包括使用H1和H2抗组胺药来控制瘙痒以及控制可能出现的胃酸过度分泌。肥大细胞病患儿的预后各不相同；大约一半的色素性荨麻疹患儿到青春期时皮损和症状可能会消退。
