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以外阴湿疹样皮损为首发表现的成人朗格汉斯组织细胞增生症 被引量:2

Genital eczema-like lesions as the first manifestation of adult Langerhans cell histiocytosis
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摘要 报告1例以外阴湿疹样皮损为首发表现且侵犯骨骼的成人朗格汉斯组织细胞增生症(LCH)。患者女,43岁。外阴红斑、丘疹、渗出伴瘙痒2年,肛周疣状增生物伴瘙痒1个半月。皮肤科检查:外阴淡红色斑块,表面有糜烂、结痂,伴轻度渗出;肛周可见疣状增生物。肛周皮损组织病理检查:真皮内组织细胞及淋巴细胞浸润。免疫组化检查示S-100蛋白及CD1a均(+)。骶髂关节CT示左侧髂骨膨大、骨质破坏、外侧缘骨皮质增厚,部分骨皮质不连续。诊断:LCH。治疗:给予沙利度胺0.1 g,每日2次口服,皮损明显好转。 A case of adult Langerhans cell histiocytosis(LCH) with eczematoid lesion on the vulva as the first manifestation is reported. A 43-year-old woman presented with itchy rash on vulva for 2 years, and perianal lesion accompanied with itching for one and a half months. Physical examination showed erythematous plaques with erosions,crusts and exudation on the vulva, and verrucous vegetation on the perianal area. Pathological examination showed histiocytic and lymphocytic infiltration in the dermis. The immunohistochemical staining showed positive for S-100 and CDla. CT of sacroiliac joint showed the left ilium expansion, bone destruction, incrassation of bone cortex on the lateral border, and a partial discontinuity of the bone cortex. The diagnosis of LCH was made. Thalidomide was given 200 mg every day, and the rash improved markedly.
出处 《临床皮肤科杂志》 CAS CSCD 北大核心 2018年第1期25-28,共4页 Journal of Clinical Dermatology
关键词 朗格汉斯组织细胞增生症 成人 Langerhans histiocytosis cell, adult
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