曲尼司特治疗泛发性肥大细胞增生病1例

Tranilast therapy for generalized cutaneous mastocytosis: a case report

报告曲尼司特治愈泛发性肥大细胞增生病1例。患儿男,6岁。面部、躯干、四肢广泛分布许多绿豆至黄豆大的斑丘疹和丘疹,部分为小结节或增生性瘢痕样皮损,明显高出皮面,呈圆形、椭圆形或不规则形,大多呈棕红色或棕褐色。组织病理检查示表皮角化过度伴角化不全,真皮浅、中层及附属器周围见致密的肥大细胞和少许嗜酸性粒细胞浸润。诊断为泛发性肥大细胞增生病。单用曲尼司特口服至第110天时,除留有少许点状色素沉着外,面部、躯干、四肢的皮损全部消退。服药期间未见不良反应。停药后随访6个月无复发。

Here is reported a case of generalized cutaneous mastocytosis treated with tranilast. A 6-year-old boy had papules and maculopapular eruption with scattered nodule and keloid-like lesions on the face, trunk and extremities. The lesions were brown or dark brown in color, bean-sized and elevated with round, oval or irregular appearance. Dariers sign was positive. Histopathological examination revealed hyperkeratosis accompanied by parakeratosis in the epidermis, dense mast cells and eosinophils infiltrates in the superficial and middle dermis and periadnexal regions, which suggested cutaneous mastocytosis. The patient was treated with oral tranilast alone for 110 days. All the skin lesions subsided with hyperpigmented spots left. Side effects were not noticed during the medication. No recurrence was found within 6-month follow-up. <