肥大细胞增生症新变异临床病理类似组织细胞和血管炎异常3例报道  

A new variant of mastocytosis: Report of three cases clinicopathologically mimicking histiocytic and vasculitic disorders

作　　者：Dunst K.M. Huemer G.M. Zelger B.G. Zelger B. 王琼 

机构地区：[1]Clinical Department of Dermatology and Venereology, Innsbruck Medical University, Anichstrasse 35, A- 6020 Innsbruck, Austria

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2005年第12期52-53,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red- brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier’ s sign). The histological hallmark of the disease is an increase in oval to spindle- shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish- yellow- brown macules of 3- 10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red- brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier' s sign). The histological hallmark of the disease is an increase in oval to spindle- shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish- yellow- brown macules of 3- 10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.

关 键 词：肥大细胞增生 血管炎 组织细胞 色素性荨麻疹 变异类型 褐色斑 皮肤附属器 临床病理类型 组织学检查 组织学特征 

分 类 号：R758.24[医药卫生—皮肤病学与性病学]