Innocenzi D, Silipo V, Giombini S, Ruco L, Bosman C, Calvieri S
Institute of the Dermatological Clinic, Università' degli Studi La Sapienza Roma, Italia.
J Cutan Pathol. 1998 Nov;25(10):563-7. doi: 10.1111/j.1600-0560.1998.tb01742.x.
Sinus histiocytosis with massive lymphadenopathy (SHML) (Rosai-Dorfman disease) is a rare self-limited histiocytic disorder of unknown origin, usually present with cervical lymphadenopathy, fever, elevated ESR and haematologic abnormalities. Extranodal lesions may occur as initial manifestations of the disease. In most cases skin lesions are associated with nodal or extranodal localization. A 54-year-old male is reported who, for a period of ten months, presented numerous slowly enlarging, rounded papular lesions on the face, neck, trunk as well as severe cervical lymphadenopathy without general and haematological disturbances. Thereafter a fungating mass developed in the nasal cavity. Histological, histochemical and immunohistochemical tests of skin, mucosal and lymph-node lesions were consistent with a diagnosis of SHML. The immunohistochemical characterization of the cytophagocytic S-100+ macrophages revealed that they were highly positive for the adhesion molecules Vitronectin receptor av/b3, which is used by tissue macrophages for removal of effete cells through phagocytosis.
伴巨大淋巴结病的窦性组织细胞增生症（SHML）（罗萨伊-多夫曼病）是一种罕见的、病因不明的自限性组织细胞疾病，通常表现为颈部淋巴结病、发热、血沉升高和血液学异常。结外病变可能是该疾病的初始表现。在大多数情况下，皮肤病变与淋巴结或结外定位相关。本文报道了一名54岁男性，在十个月的时间里，其面部、颈部、躯干出现了许多缓慢增大的圆形丘疹样病变，同时伴有严重的颈部淋巴结病，但无全身及血液学紊乱。此后，鼻腔出现了一个蕈样肿物。对皮肤、黏膜和淋巴结病变进行的组织学、组织化学和免疫组织化学检查结果与SHML的诊断相符。对吞噬细胞性S-100+巨噬细胞的免疫组织化学特征分析显示，它们对黏附分子玻连蛋白受体αv/β3呈高度阳性，组织巨噬细胞利用该受体通过吞噬作用清除衰老细胞。
