儿童组织细胞性坏死性淋巴结炎并发皮损12例

A study on 12 cases of histiocytic necrotizing lymphadenitis with rash in children

目的:提高对组织细胞性坏死性淋巴结炎及并发皮损的认识。方法:对我院12例并发皮损的组织细胞性坏死性淋巴结炎患儿的临床资料进行回顾性分析。结果:经淋巴结组织病理检查确诊的组织细胞性坏死性淋巴结炎12例,并发形态多样的皮损。其中多形红斑样损害2例,紫癜样损害3例,充血性斑丘疹7例。其中2例行皮损组织病理检查,1例表现为:表皮角化过度及角化不全,棘层不规则肥厚,表皮突下延;真皮乳头水肿,真皮浅层、小血管及皮肤附属器周围见多量核尘,大量淋巴组织细胞和中性粒细胞浸润。间接免疫荧光:IgA(-),IgG(-),IgM(-),C3(-),C4(-),fibrin(-),Clq(-)。另1例患者皮损组织病理表现为:表皮细胞水肿,部分基膜增厚,真皮见灶状淋巴细胞浸润。间接免疫荧光:基膜IgM(+),C3(+),IgA(-),IgG(-),C4(-),fibrin (-),Clq(-)。结论:组织细胞性坏死性淋巴结炎是一种良性、自限性疾病,确诊依赖于淋巴结组织病理检查。30%的患者可伴有皮损,应注意与病毒疹、系统性红斑狼疮、淋巴瘤、猫抓病和淋巴结结核等相鉴别。

Objective： To improve the recognizition of accompanied skin lesions in histiocytic necrotizing lymphadenitis. Methods： The clinical and laboratory data concerning 12 histiocytic necrotizing lymphadenitis with rash were analyzed retrospectively. Results： Twelve cases of histiocytic necrotizing lymphadenitis verified by histopathological examination were analyzed. All cases had various types of skin eruptions, 2 presented with erythema multiforme, 3 with peteehia, and 7 with erythematous papule. Histologically, one showed epidermal hyperkeratosis, parakeratosis, irregular acanthosis, cuticular process extension, edema of papillare cutis as well as plenty of nuclear dust and infiltration of many lymphocytes and neutrophils in superficial dermis, perivascular and periadnexal tissues. Immunofluorescence demonstrated IgA（-）, IgG（-）, IgM（-）, C3（-）, C4（-）, fibrin（-）, Clq（-）. Another showed epidermal cell swelling, thickening of basement membrane, and focal dermal lymphocyte in- filtration. Basement membrane was IgM （＋）, C3 （＋）, IgA（-）, IgG（-）,C4（-）, fibrin（-）, Clq（-）. Conclusions： Histiocytic necrotizing lymphadenitis is a benign and self-limiting lymphadenitis. Diagnosis is usually based on histological examinations of the affected lymph node. About 30% of patients have skin eruptions. It should be differentiated from virus infectious disease, SLE, malignant lymphoma, cat scratch disease and lymph tuberculosis.