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76岁女性患者,外阴部溃疡伴突眼5年,双侧乳房下红斑糜烂半年,尿崩症3个月。皮损为红斑、丘疹,部分表面有褐红色痂,主要位于双侧乳房下皱襞处、双腋窝及腰部。皮损组织病理检查显示真皮浅层局限性胞质丰富的大细胞和淋巴样细胞浸润,并见部分大细胞移入表皮或毛囊上皮,细胞核大深染,有异形性,核周有空晕,免疫组化显示这些大细胞S100、CD1a阳性,CD68阴性。根据患者的临床表现、组织病理和免疫组化结果,确诊为慢性多灶性朗格汉斯细胞增生症。
76-year-old female patient, vulvar ulcer with exophthalmos 5 years, bilateral breast erosion erythema six months, diabetes insipidus 3 months. Lesions of the erythema, papules, part of the surface of the maroon scab, mainly located in bilateral breast folds, double armpits and waist. Histopathological examination of the lesional dermis showed that the superficial layers of the dermis were infiltrated with macrophages and lymphoid cells with abundant cytoplasm. Some large cells were found to migrate into the epidermis or the hair follicle epithelium. The nuclei were deeply stained, irregularly shaped, These large cells S100, CD1a positive, CD68 negative. According to the patient’s clinical manifestations, histopathology and immunohistochemistry results, diagnosed as chronic multifocal Langerhans cell hyperplasia.