以泛发性丘疹为表现的成人肥大细胞增生病

Adult mastocytosis with generalized papules on the trunk and extremeties

报告成人肥大细胞增生病1例。患者男,74岁。全身泛发粟粒至绿豆大红色丘疹伴轻度瘙痒4年,皮损自行消退后形成褐色色素沉着斑点的同时不断出现新皮损,Darier征阴性,部分皮损呈紫癜样外观。组织病理学显示真皮浅层中等量肥大细胞弥漫浸润,伴部分细胞异形,诊断为肥大细胞增生病。由于其临床表现特殊,皮损与临床所见的皮肤肥大细胞增生病(包括色素性荨麻疹、弥漫型皮肤肥大细胞增生病、持久性发疹性斑状毛细血管扩张等)不同,导致长期不能确诊。该患者在随访过程中出现系统受累。

A case of adult mastocytosis is reported. The patient, a 74-year-old man, developed generalized papules 2 to 5 mm in diameter on the trunk and extremities with mild pruritus for 4 years. The papules occurred concomitantly after spontaneous remission. Pigmentation was noticed after the remission but Darier＇s sign was negative. Skin biopsy revealed moderate infiltration of mast cells in the upper dermis, some of which showed an abnormal morphology, and the diagnosis of mastocytosis was made. Due to the peculiar clinical features which were different from any of the subtypes of cutaneous mastocytosis, the patient was followed-up for a year and a half, the bone marrow was found to get involved.