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Letterer-Siwe病是组织细胞增多症中最少见、最恶性的一种变型,通常侵犯新生儿及幼小婴儿。典型表现为广泛侵犯皮肤、齿龈、内脏、骨骼及下丘脑。也可侵犯眼眶,但早期不易觉察,直至软组织浸润引起眼球突出,方被发现。本文介绍一例侵犯眼眶及其CT表现。病例为4(1/2)月男婴,患有胸部、腹部、腹股沟部红斑丘疹,伴有头皮脂溢性皮炎及双腋部溃疡性肿块,并有肛门周围溃疡及化脓性腹股沟病变。此外尚有口腔念珠菌病,出牙时牙龈过度增生,前后颈部淋巴腺病,肝肿大及右侧中耳炎。
Letterer-Siwe disease is one of the least and most malignant forms of histiocytosis and usually affects newborns and young infants. Typical manifestations of extensive violations of the skin, gums, internal organs, bones and hypothalamus. Can also violate the orbit, but not easy to detect early, infiltrating the soft tissue until the eye is prominent, was found. This article describes an example of violation of the orbit and its CT findings. Cases of 4 (1/2) months baby boy, suffering from chest, abdomen, groin erythema, with seborrheic dermatitis and double axillary ulceration, and anal ulcers and purulent groin lesions. In addition there are oral candidiasis, gums over teeth when tooth hyperplasia, cervical lymph node disease before and after, hepatomegaly and right otitis media.