1例勒雪病的临床特征和病理表现

Clinical and pathologic features of Letterer-Siwe disease: A case report and review

勒雪病是组织细胞增生症的一种类型,临床罕见,本文对收治的1例勒雪病进行报道。患儿男性,2003年7月5日因口腔溃烂、牙齿松动、脱落1月,双耳道溢脓2周就诊于四川大学华西口腔医院。检查见全身皮肤散在红色皮疹、瘀点,头皮与躯干尤为明显;双侧中耳炎;腭部及下颌牙龈出现增生性溃疡并伴有病损区牙齿的松动、脱落,X线片显示左下颌骨破坏;肝功能异常;细胞免疫功能低下伴顽固性念珠菌病。病理检查显示组织样细胞浸润,免疫组化检查:CD1a蛋白阳性,S-100蛋白染色阳性。诊断:勒雪病。本文根据本病例的特点及相关文献对勒雪病的临床特征、诊断和治疗进行了讨论。

Letterer-Siwe disease （LSD） is a kind of a histiocytosis, rarely seen in clinic. However, its prognosis is poor. A case of LSD which complaints of the oral ulcer is reported in this article. The patient of the case was a boy, came to hospital on July 5, 2003 because of oral ulcers, teeth loosing and falling off for a month, and pus running over from both antra auris for two weeks. No familial history of genetic diseases and special infections were found. The case consisted of the following features after exam： Erythematous papules and petechia dotted in the skin, primarily on scalp and trunk, two ears suffering otitis media, oral proliferative ulcers appearing in palate and lower gingival accompanying with the lower teeth loosing in the lesion area, in the meantime, the destruction of madibula on the left side also found out by X-ray, hepatic dysfunction and poor cellular immunity coming along with pertinacious candidosis. The pathologic exam showed histioid cell infiltration, and the immunohistochemical exam showed CDIa（＋）, S-100（＋）. Diagnosis defined it LSD. According to the case＇s character and literature review, the clinical features, diagnosis and therapy of LSD were discussed.